nursing care of the child with cancer
DESCRIPTION
ONCOLOGYTRANSCRIPT
NURSING CARE OF THE CHILD WITH CANCER
Pediatric Oncology
Acute leukemia Brain tumors Lymphoma Neuroblastoma Wilmrsquos tumor Rhabdomyosarcoma Retinoblastoma Osteosarcoma Ewingrsquos sarcoma
What is Leukemia
Most common childhood malignancy
Acute lymphoblastic leukemia (ALL)
Acute nonlymphoblastic leukemia (ANLL) Acute myelogenous
leukemia (AML)
Brain Tumors
2nd most common type of cancer
1200 US cases diagnosedyear
Described in terms of Metastasis (M stage) Size of tumor (T stage)
Brain Tumors
Terminal to curable
Neuropsychological impact varies based on Location size tumor
type Type of treatment Disease of complications Patient factors Social factors
Medical Treatment
Radiation Chemotherapy Surgery
Most cancers considered CURED if no relapse in 5 years
Bone Marrow Transplantation
Aggressive treatment for malignancies Give near-lethal doses
of chemotherapy or radiation
Replace dead cells with transplanted healthy cells
Autologous vs Allogenic
Stages of BMT
Donor search amp initial evaluation
Preparative treatment
Bone marrow infusion
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Pediatric Oncology
Acute leukemia Brain tumors Lymphoma Neuroblastoma Wilmrsquos tumor Rhabdomyosarcoma Retinoblastoma Osteosarcoma Ewingrsquos sarcoma
What is Leukemia
Most common childhood malignancy
Acute lymphoblastic leukemia (ALL)
Acute nonlymphoblastic leukemia (ANLL) Acute myelogenous
leukemia (AML)
Brain Tumors
2nd most common type of cancer
1200 US cases diagnosedyear
Described in terms of Metastasis (M stage) Size of tumor (T stage)
Brain Tumors
Terminal to curable
Neuropsychological impact varies based on Location size tumor
type Type of treatment Disease of complications Patient factors Social factors
Medical Treatment
Radiation Chemotherapy Surgery
Most cancers considered CURED if no relapse in 5 years
Bone Marrow Transplantation
Aggressive treatment for malignancies Give near-lethal doses
of chemotherapy or radiation
Replace dead cells with transplanted healthy cells
Autologous vs Allogenic
Stages of BMT
Donor search amp initial evaluation
Preparative treatment
Bone marrow infusion
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
What is Leukemia
Most common childhood malignancy
Acute lymphoblastic leukemia (ALL)
Acute nonlymphoblastic leukemia (ANLL) Acute myelogenous
leukemia (AML)
Brain Tumors
2nd most common type of cancer
1200 US cases diagnosedyear
Described in terms of Metastasis (M stage) Size of tumor (T stage)
Brain Tumors
Terminal to curable
Neuropsychological impact varies based on Location size tumor
type Type of treatment Disease of complications Patient factors Social factors
Medical Treatment
Radiation Chemotherapy Surgery
Most cancers considered CURED if no relapse in 5 years
Bone Marrow Transplantation
Aggressive treatment for malignancies Give near-lethal doses
of chemotherapy or radiation
Replace dead cells with transplanted healthy cells
Autologous vs Allogenic
Stages of BMT
Donor search amp initial evaluation
Preparative treatment
Bone marrow infusion
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Brain Tumors
2nd most common type of cancer
1200 US cases diagnosedyear
Described in terms of Metastasis (M stage) Size of tumor (T stage)
Brain Tumors
Terminal to curable
Neuropsychological impact varies based on Location size tumor
type Type of treatment Disease of complications Patient factors Social factors
Medical Treatment
Radiation Chemotherapy Surgery
Most cancers considered CURED if no relapse in 5 years
Bone Marrow Transplantation
Aggressive treatment for malignancies Give near-lethal doses
of chemotherapy or radiation
Replace dead cells with transplanted healthy cells
Autologous vs Allogenic
Stages of BMT
Donor search amp initial evaluation
Preparative treatment
Bone marrow infusion
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Brain Tumors
Terminal to curable
Neuropsychological impact varies based on Location size tumor
type Type of treatment Disease of complications Patient factors Social factors
Medical Treatment
Radiation Chemotherapy Surgery
Most cancers considered CURED if no relapse in 5 years
Bone Marrow Transplantation
Aggressive treatment for malignancies Give near-lethal doses
of chemotherapy or radiation
Replace dead cells with transplanted healthy cells
Autologous vs Allogenic
Stages of BMT
Donor search amp initial evaluation
Preparative treatment
Bone marrow infusion
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Medical Treatment
Radiation Chemotherapy Surgery
Most cancers considered CURED if no relapse in 5 years
Bone Marrow Transplantation
Aggressive treatment for malignancies Give near-lethal doses
of chemotherapy or radiation
Replace dead cells with transplanted healthy cells
Autologous vs Allogenic
Stages of BMT
Donor search amp initial evaluation
Preparative treatment
Bone marrow infusion
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Bone Marrow Transplantation
Aggressive treatment for malignancies Give near-lethal doses
of chemotherapy or radiation
Replace dead cells with transplanted healthy cells
Autologous vs Allogenic
Stages of BMT
Donor search amp initial evaluation
Preparative treatment
Bone marrow infusion
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Stages of BMT
Donor search amp initial evaluation
Preparative treatment
Bone marrow infusion
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Stages of BMT
Severe neutropenia
Engraftment Graft-versus-Host
disease
Follow-up
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Phases of Cancer
Diagnosis Initiation of treatment Remission or illness stabilization Completion of medical therapy Long-term survival and cue vs Relapse
or deterioration Terminal illness amp death Post-death adjustment of family
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Diagnosis
Address emotional reaction
Evaluate family understanding
Determine financial resources Financial Social
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Diagnosis
Communication with others What to tell the child
1 Go slowly
2 Encourage questions
3 Convey hope
4 Establish trust
5 Gauge details to developmental ability
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Treatment
Disruption of life Complex treatment
schedules Feeling poorly Reaction of others Maintain contact with
school
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Treatment Coping with acute amp chronic pain
Bone marrow aspirations (BMA) amp lumbar punctures (LP)
Distraction relaxation hypnosis Anticipatory nausea amp vomiting
Classical conditioning Relaxation imagery distraction
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Treatment
Parents need to feel some control during the treatment process Helplessness Hopelessness
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Donrsquot forget the siblings
Suggestions for parents Give them time too Choose caregivers
carefully Set limits on gifts Allow them to ldquohelp
outrdquo Answer questions
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Coping Strategies
Adaptive Positive reframing Acceptance Social support Maintaining objectivity Active involvement
Maladaptive Denial Helplessness Cognitive escape Behavioral escape
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Remission or Stabilization
Maintenance chemotherapy
Return to school Social re-entry concerns Academic performance
Role of doubts and fears
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Completion of Treatment
Emotional reliance on treatment
Weaning from frequent appointments
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Completion of Treatment
Marital stress
Difficulty with discipline
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Long-term Survival amp Care
Learning amp memory problems
Endocrine dysfunction
Emotional outcomes
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Relapse amp Recurrence
Occurs in 40-50 of pediatric oncology patients
May be harder emotionally than initial diagnosis
Re-learn basic info Experimental treatments etc
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
BONE TUMOR
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
A bone tumor is a neoplastic growth of tissue in bone Abnormal growths found in the bone can be either benign or malignant
Bones are classified according to their shape- Long bone Flat bone Short bone
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Long bone anatomy Diaphysis long shaft of bone Epiphysis ends of bone Epiphyseal plate growth plate Metaphysis bw epiphysis and diaphysis Articular cartilage covers epiphysis Periosteum bone covering (pain sensitive) Sharpeyrsquos fibers periosteum attaches to
underlying bone Medullary cavity Hollow chamber in bone
- red marrow produces blood cells- yellow marrow is adipose
Endosteum thin layer lining the medullary cavity
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Diaphysis Epiphysis
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Metaphysis
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Histology of bone tissueCells are surrounded by matrix
- 25 water- 25 protein- 50 mineral salts
4 cell types make up osseous tissueOsteoprogenitor cellsOsteoblastsOsteocytesOsteoclasts
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoprogenitor cells
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into osteoblasts
- found on inner surface of periosteum and endosteum
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoblasts
- bone forming cells- found on surface of bone- no ability to mitotically divide - collagen secretors
Osteocytes
- mature bone cells- derived form osteoblasts- do not secrete matrix material- cellular duties include exchange of nutrients and waste with blood
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoclasts- bone resorbing cells- bone surface- growth maintenance and bone repair
Abundant inorganic mineral salts- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate CaCO3
- Magnesium Hydroxide Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoprogeniter cells ampOsteoblast
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoclast
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Precursors of malignancy in bone
High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Classification of primary tumour involving bones
Histological type
Benign Malignant
Hematopoietic Myeloma
Malignant lymphoma
Chondrogenic Osteochodroma chondrosarcoma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Histological type
Benign Malignant
Osteogenic Osteoma Osteosarcoma
Osteoid osteoma
osteoblastoma
Unknown origin Giant cell tumour
Ewing tumour
Giant cell tumour
admantinoma
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Histological type
Benign Malignant
Histiocytic origin
Fibrous histiocytoma
MFH
Fibrogenic Metaphyseal fibrous defect
Dysplastic fibroma
Fibrosarcoma
Notochordal Chordoma
Vascular Hemangioma Hemangioendothelioma
Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Distribution of bone tumors in long bones
Epiphyseal lesions Chondroblastoma Giant cell tumor
Metaphyseal intramedullary lesions Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Metaphyseal lesions centered in the cortex
Nonossifying fibroma (NOF) Osteoid osteoma
Metaphyseal exostosis Osteochondroma
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Diaphyseal intramedullary lesions Ewingrsquos sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma Diaphyseal lesions centered in the cortex Adamantinoma Osteoid osteoma
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Sites of Tumors
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Important Facts 0001 of all cancers MC benign tumor--- Osteochondroma Osteoid
Osteoma MC Skeletal malignancyndash Metastasis MC Bone tumor in Pediatric age group amp adults-
Osteosarcoma MC in lt 10 y--- Ewingrsquos sarcoma MC Primary bone tumor ndash Multiple Myeloma
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
PRESENTING SYMPTOMS Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problemPAIN- is most frequent symptom MASS- rate of enlargement is important -Fluctuating mass can be cystganglion or hemangioma -Family HO masses near the joint may be indicator of Ollierrsquos disease or
Maffucci SyndromeNEUROLOGICAL SYMPTOM- found in few patients such as sacral tumors amp with
tumors located near the nerve causing compression of nerveespecially common in sciatic notch inguinal canal amp popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY- found in pelvic tumors which are painless amp without a palpable mass amp cause swelling due to
compression of iliac vein
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
PHYSICAL EXAMINATION Evaluation of patientrsquos general health TUMOR MASS should be measured amp its locationshape
consistencymobilitytendernesslocal temp amp change with position should be noted
SKIN amp SUBCUTANEOUS TISSUE Small dialated superficial veins overlying the mass are
produced by large tumorsCafeacute-au-lait spots amp subcutaneous neurofibromas indicate
Von Recklinghausenrsquos diseaseA venous malformation Maffucci SyndromeREGIONAL LYMPH NODES sign of metastatic diseaseAtrophy of surrounding musculature should be
recordedalso neurological deficits amp adequacy of circulation
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
HISTORY OF THE PATIENT
AGE- most imp informationbcoz of their presentaion in sp age group
1st decade- usually ABC SBC2nd decade-ChondroblastomaosteosarcomaEwings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma SEX- less imp than age RACE- little imp Ewings rare in african descent HO any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma Various chemical carcinogens- zinc beryllium silicate beryllium
oxide Currently the most worrisome amp controversial is Nickel which is
used in many orthopedic devices
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
LABORATORY TEST Alkaline phosphatase ndash Higher
PTH test Lower
Serum phosphorus Higher
Ionized calcium and serum calcium Higher
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan
uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone
PET- uses radioactive glucose to locate cancer This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
BIOPSY The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign the bone cancer type (primary or secondary bone cancer) and stage
According to the tumor size and type (malignant or benign) and the biopsys purpose (to remove the entire tumor or only a small tissue sample) there are two types of biopsies used in bone cancer diagnosis These are needle biopsy and incisional biopsy
1 Needle biopsy During this procedure a small hole is made in the affected bone and a tissue sample from the tumor is removed
There are two types of needle biopsies Fine needle aspiration During this procedure the tissue
sample is removed with a thin needle attached to a syringe Core needle aspiration During this procedure the surgeon
removes a small cylinder of tissue sample from the tumor with a rotating knife like device
2 Incisional biopsy During this procedure the surgeon cuts into the tumor and removes a tissue sample
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
BONE FORMING TUMOURS
Benign Osteoma Osteoid Osteoma
Benign Aggressive Osteoblastoma
Malignant Osteogenic Sarcoma
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoma
Benign asymptomatic slow-growing osteogenic lesion
AgeSex 40-50 yr MF = 21 Bones involved flat bones of the skull
and face may protrude in the paranasal sinus
Parosteal location Gardnerrsquos syndrome
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Ivory like bony mass
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Figure 2 The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling Figure 3 Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoid osteoma
SignsSymptoms Pain characteristically more intense at night relieved by NSAID
and eliminated by excision Scoliosis
Age 10-30 years
Sex M gt F (21)
Anatomic Distribution Nearly every location most frequent in femur tibia( Over 50)
humerus bones of hands and feet vertebrae and fibula Metaphysis Diaphysis (cortical) of long bones Vertebral lesions may be associated with scoliosis
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Small central osteolytic nidus surrounded by dense bone
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
The small reddish central nidus is surrounded by a thick layer of sclerotic bone
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Wedge shaped nidus which is surrounded by dense sclerotic bone
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
New osteoid and bone formation by plump osteoblasts The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoid osteoma with anastomosing trabeculae of woven bone
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoblastoma Also called as Giant osteoid osteoma
Osteoblastoma is similar to osteoid osteoma with more aggressive behavior
DD from osteoid osteoma- Pain
Absence of reactive bone Large size
Location In spine or major bones of lower extremity
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Well differentiated radiopaqueradiolucent lesion
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoblastoma-The histologic appearance is
identical to that of osteoid osteoma
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Recurrent osteoblastoma-The appearance is similar to that of osteoid osteoma
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteosarcoma
Most frequent primary malignant bone tumour
AgeSex 10-25 yrs amp gt40 MF = 32 Predisposing conditions
Pagetrsquos disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Codmanrsquos triangle ldquoSunray ldquo appearance
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Tumor is located at the typical metaphyseal site The tumor shown in A is largely restricted to bone whereas that illustrated in B is accompanied by massive soft tissue extension
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
lsquoskip metastasisrsquo located in the upper half of the femur The primary tumor was located in the lower metaphysis of the same bone
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Lace-like osteoid deposition is very characteristic of this neoplasm
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Microscopic variants
Telangiectatic
Blood filled cystic space and thus radiologically appears as pure lytic lesion
Pathological fractures Grossly the lesion simulate aneurysmal bone
cyst Detection of malignant stroma in the septa
that separate the bloody cysts
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Telangiectatic osteosarcoma
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Telangiectatic osteosarcoma A The low-power architecture closely simulates the appearance of an aneurysmal bone cystB Malignant osteoid is present in the septa
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Telangiectatic osteosarcoma Spaces containing blood are separated by septa The cells appear malignant even at this level of magnification
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Fibrohistiocytic
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Small cell variant
Uniform small size tumour cells Diffuse pattern of growth Simulate Ewingrsquos sarcoma and
malignant lymphoma
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Anaplastic
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Well differentiated intramedullary
This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion
In contrast to fibrous dysplasia
1- this tumor shows radiographic evidence of cortical destruction
2-Microscopically atypia is minimal but still present
3-The invasive growth pattern
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Variants defined on the basis of topographic clinical and radiographic features
Juxtacortical (parosteal) Slightly older age group Juxtacortical position in the metaphysis of long
bones
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Juxtacortical osteosarcoma of upper femur There is only minimal involvement of the cortex
Juxtacortical osteosarcoma large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia and the bone appears to arise directly from the spindle cells
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Periosteal osteosaocoma Grows on surface of long bones Upper tibial shaft or femur
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Periosteal osteosarcoma The white shining appearance is due to the high content of cartilage
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
periosteal chondrosarcoma There is a predominance of myxochondroid areas
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteosarcoma of jaw Patients affected are slightly older (average age 34
years) And most lesions show a prominent chondroblastic
component The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla
Osteosarcoma in Pagetrsquos disease Osteosarcoma are of the polyostotic type Pelvis humerus femur tibia amp skull Large number of osteoclasts alternating with atypical
osteoblast
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Histochemistry IHC amp molecular genetics
Strong alkaline phosphatase activity Vimentin S-100 keratin amp EMA Osteonectin osteocalcin osteopontin
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Prognostic factors
Pagetrsquos disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2neu expression P-glycoprotein
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
CARTILAGE FORMING TUMOURS
Benign Enchondroma Peri-osteal Chondroma Osteochondroma
Benign Aggressive Chondromyxoid Fibroma Chondroblastoma
Malignant Chondrosarcoma
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondroma
Benign tumor of mature hyaline cartilage Age ndash 20-50 yrs Usually solitary30 are multiple Bones involved small bones of hand amp feet Asymptomatic pain amp swelling
Enchondroma is the most common tumor of the bones of the hand
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Enchondromas
Begin in spongiosa of diaphysis from which they expand and thin cortex
Unusual in ribs and long bones
Juxtacortical Chondroma
Much less common than enchondroma Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
2 syndromes characterized by multiple chondromas
Ollierrsquos disease
Maffuccirsquos syndrome
Both disorders have 25 risk of malignant transformation to chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Enchondroma-The tumor has a typical lobulated appearance
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Osteochondroma
Also known as exostosis Most frequent benign cartilaginous
tumour Agesex - lt20yr MF=31 Bones involved lower femur upper tibia
upper humerus and pelvis Location Metaphysis
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Probably not a true neoplasm
Inactivation of both copies of the EXT gene in the growth plate chondrocytes
Presents as slow growing mass painful
lt1 cases show malignant transformation
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
A- Large osteochondroma of femur with a bilobed appearance B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Projection with cortex continuous with underlying bone may be pedunculated cartilaginous cap with
frequent calcification
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Microscopic-Mature bone is covered by a well-
differentiated cartilaginous cap
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Microscopic
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondroblastoma
Rare benign cartilage producing tumour AgeSex 2nd decade MF=21 Bones involved Distal femur proximal
humerus and tibia Location Epiphysis Pain is constant
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Typical sharply delineated lytic appearance of chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Gross appearance of chondroblastoma of upper end of the humerus associated with aneurysmal bone cyst-like changes
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondroblastoma A- Small tumor cells of round shape are accompanied by scattered osteoclasts B-Immunoreactivity for S-100 protein in the neoplastic component
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondroblast Prominent Indented NucleusEosinophilic Cytoplasm Thick Cell MembraneUniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondroblastoma with eosinophilic chondroid matrix giant cells and mononuclear cells
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Imagine the cells present without the nuclei The thickened cell membranes would give a chicken wire fence appearance
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondromyxoid Fibroma
Benign tumour of cartilaginous origin AgeSex 20-30 yr MF=21 Bones involved Long bonesgtflat bones
gtvertebrae Location Metaphysis Localised pain with or without
tenderness
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance in which myxochondroid islands alternate with more cellular foci
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondrosarcoma
Second most common malignant tumour of bones
Arise de novo or from pre-existing benign cartilagenous tumour
Divided into two major categories
Conventional chondrosarcoma Chondrosarcoma variants
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Conventional chondrosarcoma
30 ndash 60 yr of age MgtF Divided according to location
Central
Peripheral
Juxtacortical
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
-Typical chondrosarcoma of femurI-ill defined margins fusiform thickening of shaft perforation of cortex
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Typical chondrosarcoma
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Peripheral Variant Tumour is present on the surface of
bone May arise de-novo or from cartilaginous
cap of preexisting osteochondroma
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Juxtacortical(periosteal) variant Location
shaft of long bone (most often femur) Cartilaginous lobular pattern with areas
of spotty calcification endochondral ossification
Closely related to periosteal osteosarcoma
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Microscopic
Wide range of differentiation and graded into well differentiated moderately differentiated poorly differentiated
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Well-differentiated chondrosarcoma The tumor has a distinctly lobulated quality
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Well differentiated- High-power appearance of grade 1 chondrosarcoma A few doubly nucleated cells and moderate atypia
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Poorly differentiated
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Grading system
Grade I lesions contain hyaline cartilage manifested by sparse cellularity The cells typically contain dark pyknotic nuclei lt20 of cells contain large nuclei and fine nuclear chromatin Mitosis is absent
Grade II A) lesion are slightly more cellular and gt20 nuclei are larger than nucleus of mature lymphocyte Binucleate cells are easily found Mitosis is absent
B) cellular lesions with numerous binucleated cells and nuclear atypia Mitosis is present but not more than
1 10hpf Grade III Mitosis atleast gt=2 10 hpf
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma
Features consistent with chondrosarcoma arePain attributable to lesion Age greater than 50Cortical destruction and a soft tissue massPeriosteal reaction and thickeningEndosteal erosiongt23 cortical thickness on a CT scanSize greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondrosarcoma variants
Dedifferentiated chondrosarcoma Worst prognosis Agesex sixth decade MF =11 Bones involved pelvis femur Poorly differentiated sarcomatous
component at periphery of otherwise typical low-grade chondrosarcoma usually central type can be peripheral
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Microscopic
Dedifferentiation may be in initial lesion more often in specimens from recurrent
tumor microscopic appearance of this component
may be rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Clear cell variant Behaves as low-grade malignancy Can undergo dedifferentiation AgeSex 30-40 yrsMF= 251 Location proximal end of femur and
humerus
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Clear cell chondrosarcoma with faint lobulation woven bone and clear cells
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Mesenchymal variant
Usually second or third decade of life Great variability in clinical course Location
most commonly jaw pelvis femur ribs spine
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Shows an island of well-differentiated cartilage in the center
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Cellular hemangiopericytoma-like component
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
OSTEOMA
FACIAL BONE
40-5021 MINERALIZED COMPACT BONE
OSTEOID OSTEOMA
CORTEX OF LB
10-3021 ldquoNIDUSrdquo OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE
OSTEOBLASTOMA
VERTEBRAECORTEX OF LB
10-3021 IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS
OSTEOSARCOMAA-CONVENTIONAL B-LOW GRADE CENTRAL
METAPHYSIS OF LB
10-2532
A-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS
-B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
C-TELANGIECTATIC
METAPHYSIS
BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID
D-PAROSTEAL
CORTEX OUTSIDE PERIOSTEUM
30-60 YRS MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
E-PERIOSTEAL
CORTEX INSIDE PERIOSTEUM
ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
TUMOUR LOCATION
AGEMF SALIENT PATHOLOGIC FINDING
CHONDROMA
HANDS FEETRIBS FEMUR
10-4011 VARIABLY CELLULAR HYLINE CARTILAGE
OSTEOCHONDROMA
METAPHYSISOF LBS
10-3011 CARTILAGE CAPPED BONY PROTRUSION
CHONDROBLASTOMA
EPIPHYSISOF LBS
10-2021 CHONDROID LIKE MATRIX S-100 POSITIVE CELLS WITH GROOVED NUCLEI
CHONDROMYXOID FIBROMA
METAPHYSIS OF LBS
10-3011 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
TUMOUR LOCATION AGEMF SALIENT PATHOLOGIC FINDING
CHONDROSARCOMA
PELIVIS RIBS FEMUR
31
A- CONVENTIONAL
METAPHYSIS
20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE
B-DEDIFFERENTIATED
METAPHYSIS
gt30 CONVENTIONAL CHONDROSARCOMA+HIGH GRADE SPINDLE CELL SARCOMA
C- MESENCHYMAL
METAPHYSIS
20-50 UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE
E- CLEAR CELL
EPIPHYSIS
20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Other Childhood Neoplasms
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
NEUROBLASTOMA
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullMost common extra cranial solid tumor of childhoodbullOver half of the children present with metastatic diseasebullArise from cells of the neural crest that form the adrenal medulla and sympathetic ganglia
INTRODUCTION
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullAdrenal glandbullSympathetic chainbullNeckbullThoraxbullRetroperitoneumbullPelvis
SITES
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bull8 to 10 of all childhood cancersbull10 cases per 1 million live birthsbullMost common malignant tumorof infancybullMedian age at diagnosis of 19 monthsbull(Brodeurand Maris 2006)bullThere are no geographic or racial variations
INCIDENCE
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullAutosomal dominant pattern of inheritancebull(Knudson and Strong 1972a Robertson et al 1991)bullIn familial cases median age decresesto 9 monthsbullHereditary neuroblastomapredisposition gene chromosome 16p12-13bull (Maris et al 2002)bullAmplification of the N-MYC oncogene seen in roughly 20bull(Look et al 1991 Muraji et al 1993)bullDeletion of the short arm of chromosome 1bull(Brodeur et al 1992 Caron et al 1996)
GENETICS
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bull Homer-Wright pseudorosettes- consist of eosinophilic neutrophils surrounded by neuroblasts bull Schwann cell-Other type
PATHOLOGY
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
1048711Abdominal pain1048711Palpable mass1048711fixed hard abdominal mass1048711Bone or joint pain1048711Periorbitalecchymosis1048711Cough1048711Dyspnea1048711Neurologic deficits1048711Urinary retention1048711Constipation1048711Paraneoplasticsyndromes1048711Paroxysmal hypertension1048711Palpitations1048711Flushing1048711Headache1048711Severe watery diarrhea1048711Hypokalemia1048711Acute myoclonic encephalopathy
CLINICAL FEATURES
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullLaboratory EvaluationbullRoutine InvestigationsbullHaemoglobin-Anemiain bone metsbullVanillylmandelicacid (VMA)-24 hour Urinary and SerumbullHomovanillicacid (HVA)bullTwo bone marrow aspirates and two biopsies
DIAGNOSIS
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullImagingbullUltrasound-First line Detects incidentalomabullPlain radiographs-calcified abdominal or posterior mediastinalmassbullComputed Tomography-local extent of the primary tumors Invasion of the renal parenchymabullMagnetic resonance imaging-evaluation of intraspinaltumorextension demonstrating the relationship between the major vessels and the tumorbullRadionuclide bone scanbullMeta-iodobenzylguanidinescan
DIAGNOSIS
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullSurgerybullChemotherapybullRadiation therapy
TREATMENT
bullGoalsbullEstablish the diagnosisbullStage the tumorbullExcise the tumor(if localized)bullProvide tissue for biologic studies
SURGERY
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullSurgical excisionbullChildren with stage I neuroblastomahave a disease-free survival rate of greater than 90 after exicisionbullLow-Risk Disease (Stages I II and IV-S)bullComplete excision should be undertaken only when there is not a concern for undue morbidity to vital organs or the patientbullSacrifice of major organs such as the kidney or spleen should be avoided especially in children less than one year of age
SURGERY
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullAbdominal tumors-generous transverse incisionbullLigation of feeding vessels TumorexcisedbullLymph node samplingbullnoncontiguousnodes above and below the tumorbullLiver biopsy indicated if Stage 4SbullPatients with incomplete resection initially-delayed attempt at resection of residual tumoris undertaken at the end of induction chemotherapybullSurgery is not indicated for those patients who have progressive disease at this time
SURGERY
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullThoracic tumors-posterior-lateral thoracotomybullDumbbell-shaped tumorsthat enter the neural foramina are generally treated initially with chemotherapy
SURGERY
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullAtelectasisbullInfectionbullIleusbullHaemorrhage
COMPLICATIONS
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullLocal controlbullstage IV or bulky stage III tumorsbull(Matthayet al 1989 Castleberry 1991 Evans et al 1996)bullDose-15 and 30 GybullIntraoperative radiation therapy-unresectable disease
RADIOTHERAPY
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
bullChemotherapy bullReserve laminectomy for children with progressive neurologic deterioration (Katzensteinet al 2001) bullRadiotherapy-avoided because of its adverse effect on growth of the spine
SPINAL CORD COMPRESSION
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
RHABDOMYOSARCOMA
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
What is Rhabdomyosarcoma
Rhabdomyosarcoma is a cancerous malignant tumor of the muscles that attach to bone
Rhabdomyosarcoma is the most common soft tissue cancer in children
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Where does Rhabdomyosarcoma occur
It can occur in many places in the body Some of thesemay include the head or neck the urogenital tract or the arms or legs
Depending on where the tumor(s) isare located the symptoms can vary For example tumors around the eye may cause bulging of the eye problems with vision swelling or pain While muscle tumors in the arms or legs may lead to a painful lump and are often thought to be an injury from play
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Who can get Rhabdomyosarcoma
Rhabdomyosarcoma is most commonly found in children
Usually by the time the cancer is found it is at a higher stage because this is a type of cancer with little or no symptoms in earlier stages
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
When can Rhabdomyosarcoma be treated
It is very important to begin treatment right away This is mainly because of the ability this cancer has to spread especially since it usually is not found early on
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
How is Rhabdomyosarcoma found There are many tests that are done to
diagnose Rhabdomyosarcoma
bull Some of these tests includebull Chest X-Raybull CT Scanbull Biopsy of the tumorbull Bone Scanbull Bone Marrow biopsybull Ultrasound
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
How is Rhabdomyosarcoma treated Once found treatment of Rhabdomyosarcoma is very aggressive There are many different protocols depending on the stage and location of the cancer and the age of the patient
For example a protocol for a four year old patientwith tumors in the leg and chest may be
54 weeks or 20 cycles of Chemotherapy and 53 Radiation treatments
This would also include surgery to remove the mass in the leg after the initial chemotherapy treatment but BEFORE Radiation therapy
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
How is Rhabdomyosarcoma treated (cont) Standard treatment is done by what is called VAC therapy
VAC is a combination of the drugs Vincristine Dactinomycin Cyclophosphamide
A more detailed protocol is ARST8P1 This consists of the standard VAC therapy plus the use of the following drugs
Ifosfamide Etoposide Doxorubicin Irinotecan
This ldquococtailrdquo is described as High Intensity Chemotherapy
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Why does Rhabdomyosarcoma occur
While researchers have yet to determine why this cancer occurs or what exactly causes it there is another ldquoWhyrdquo question I would like to answer ldquoWhy did I choose Rhabdomyosarcomardquo
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Meet Dorian
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
Dorian is a 5 year old patient at the pediatric office where I work
On April 15 2012 Dorian came into the office complaining of pain in his right leg After the doctor examined him she came out of the exam room and into the nurses station where I happened to be standing She looked at me and said ldquoI really hope this isnrsquot cancer I will never forget that feeling as long as I liverdquo (she was referring to the feeling of the mass on Dorianrsquos right calf she compared it to the feeling of a rubber chicken) my heart sank and from that moment on before we even knew anything Dorian had my heart On April 19 2012 we got the results from all of the tests Stage 4 Alveolar Rhabdomyosarcoma At 4pm that afternoon as I was walking out the door to go home Dorian was walking in with his mom and dad They had no idea what was coming but I did I smiled and said hello to Dorian and continued out to my car I couldnrsquot help but cry ldquoHow could this happen to such a sweet innocent little boyrdquo ldquoWhy Dorianrdquo I said to myself I will never forget that day As Irsquom sure his parents wonrsquot either
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-
As of April 25 2013 I am happy to report that Dorian is doing well and totally kicking cancers butt He has officially completed all in patient treatments and now only has to go to clinic for the last of his treatment While the road has had many ups and downs Dorian has faced them all like a champ He is the strongest kid I know and his mom is absolutely amazing I give her so much credit for the strength she has had for Dorian through this very difficult but also very rewarding past year To see where Dorian was just a few short months ago and look at him today is an absolute miracle and I am so happy to say I know this kid He certainly is one of a kind Keep kickin butt D you got this
- NURSING CARE OF THE CHILD WITH CANCER
- Pediatric Oncology
- What is Leukemia
- Brain Tumors
- Brain Tumors (2)
- Medical Treatment
- Bone Marrow Transplantation
- Stages of BMT
- Stages of BMT (2)
- Phases of Cancer
- Diagnosis
- Diagnosis (2)
- Treatment
- Treatment (2)
- Treatment (3)
- Donrsquot forget the siblings
- Coping Strategies
- Remission or Stabilization
- Completion of Treatment
- Completion of Treatment (2)
- Long-term Survival amp Care
- Relapse amp Recurrence
- Slide 23
- Slide 24
- Long bone anatomy
- Diaphysis
- Metaphysis
- Slide 28
- Slide 29
- Slide 30
- Slide 31
- Osteoprogeniter cells ampOsteoblast
- Osteoclast
- Precursors of malignancy in bone
- Slide 35
- Classification of primary tumour involving bones
- Slide 37
- Slide 38
- Distribution of bone tumors in long bones
- Slide 40
- Slide 41
- Sites of Tumors
- Important Facts
- PRESENTING SYMPTOMS
- PHYSICAL EXAMINATION
- HISTORY OF THE PATIENT
- LABORATORY TEST
- INVESTIGATIONS
- BIOPSY
- BONE FORMING TUMOURS
- Osteoma
- Ivory like bony mass
- Figure 2 The lesion consists of dense and lamellar cortical
- Osteoid osteoma
- Slide 55
- The small reddish central nidus is surrounded by a thick layer
- Slide 57
- New osteoid and bone formation by plump osteoblasts The stroma
- Osteoid osteoma with anastomosing trabeculae of woven bone
- Osteoblastoma
- Well differentiated radiopaqueradiolucent lesion
- Osteoblastoma-The histologic appearance is identical to that o
- Recurrent osteoblastoma-The appearance is similar to that of o
- Osteosarcoma
- Slide 65
- Slide 66
- Slide 67
- Slide 68
- The malignant bone is more basophilic and has more irregular bo
- Osteosarcoma showing characteristic basophilic thin trabeculae
- Lace-like osteoid deposition is very characteristic of this neo
- Slide 72
- Osteoblastic osteosarcoma with finely ramifying matrix between
- Microscopic variants
- Telangiectatic osteosarcoma
- Telangiectatic osteosarcoma A The low-power architecture clos
- Telangiectatic osteosarcoma Spaces containing blood are separa
- Slide 78
- Slide 79
- Anaplastic
- Well differentiated intramedullary
- Slide 82
- Slide 83
- Slide 84
- Parosteal osteosarcoma-The spindle cells demonstrate minimal at
- Slide 86
- Periosteal osteosarcoma The white shining appearance is due to
- Slide 88
- Bone formation in the center of a cartilaginous lobule in perio
- Slide 90
- Histochemistry IHC amp molecular genetics
- Prognostic factors
- CARTILAGE FORMING TUMOURS
- Chondroma
- Slide 95
- Slide 96
- Maffucci syndrome-Innumerable chondromas are seen concentrated
- Juxta-cortical - The tumor produces a semispherical expansion o
- Enchondroma-The tumor has a typical lobulated appearance
- Osteochondroma
- Slide 101
- A- Large osteochondroma of femur with a bilobed appearance
- Slide 103
- Microscopic-Mature bone is covered by a well-differentiat
- Microscopic
- Chondroblastoma
- Slide 107
- Gross appearance of chondroblastoma of upper end of the humerus
- Chondroblastoma A- Small tumor cells of round shape are accomp
- Chondroblast Prominent Indented Nucleus Eosinophilic Cytoplasm
- Chondroblastoma with eosinophilic chondroid matrix giant cells
- Imagine the cells present without the nuclei The thickened ce
- Chondromyxoid Fibroma
- Slide 114
- Chondromyxoid fibroma- (A) An irregularly shaped hypocellular c
- Chondrosarcoma
- Conventional chondrosarcoma
- Slide 119
- (2)
- Slide 121
- Peripheral chondrosarcoma of femur resulting in a huge exophyt
- Slide 123
- Microscopic
- Well-differentiated chondrosarcoma The tumor has a distinctly
- Well differentiated- High-power appearance of grade 1 chondrosa
- Moderately differentiated - High-power appearance of grade 2 ch
- Poorly differentiated
- Grading system
- Slide 130
- Chondrosarcoma variants
- Gross appearance of dedifferentiated chondrosarcoma of pelvic b
- Microscopic (2)
- Slide 134
- Chondrosarcoma is juxtaposed with high-grade malignant fibrous
- Slide 136
- Clear cell chondrosarcoma with faint lobulation woven bone an
- Slide 138
- Slide 139
- Cellular hemangiopericytoma-like component
- Slide 141
- Slide 142
- Slide 143
- Slide 144
- Slide 145
- Slide 146
- Slide 147
- Slide 148
- Slide 149
- Slide 150
- Slide 151
- Slide 152
- Slide 153
- Slide 154
- Slide 155
- Slide 156
- Slide 157
- Slide 158
- Slide 159
- Slide 160
- Slide 161
- Slide 162
- Slide 163
- Slide 164
- Slide 165
- Slide 166
- Slide 167
- Slide 168
- Slide 169
- Slide 170
- Slide 171
- Slide 172
- Slide 173
- Slide 174
- Slide 175
- Slide 176
- Slide 177
-