pediatric malignancies

Pediatric Malignancies

Jan Bazner-Chandler

CPNP,MSN, CNS, RN

Pediatric Malignancies 1% of all cancers

Involves tissues of:

CNS, bone, muscle, endothelial tissue

Grows in a short period of time

Causes Genetic alteration

Environmental influences

No know prevention

Metastasic disease seen in 80%

Response to Treatment Very responsive to chemotherapy

More than 60% cure rate

Classification of Tumors Embryonal tumor arises from embryonic tissue

Lymphomas = lymphatic tissue

Leukemias = blood

Sarcoma = seen in bone, cartilage, nerve and fat

Cardinal Signs of Cancer Unusual mass or swelling Unexplained paleness and loss of energy Spontaneous bruising Prolonged, unexplained fever Headaches in morning Sudden eye or vision changes Excessive – rapid weight loss.

Diagnostic Tests X-ray Skeletal survey CT scan Ultrasound MRI Bone marrow aspiration

Biopsy Identify cell to determine type of treatment

Treatment Modalities Determined by:

Type of cancer

Location

Extent of disease

Surgery The oldest form of cancer treatment Surgery plays important role in initial

diagnosis: biopsy of primary tumor. Excision of tumor when possible Facilitating treatment: insertion of catheters

for long-term treatment

Radiation Therapy The use of ionizing radiation to break apart bonds

within a cell causing cell damage and death. External beam therapy accounts for the majority of

radiation treatments in children. Problems: radiation beams cannot distinguish

between malignant cells and healthy cells.

Chemotherapy Primary treatment modality used to cure

many pediatric cancers. Chemotherapy is the use of drugs to destroy

cancer cells. The destruction is accomplished by inhibiting

cells within the body to divide, which eventually leads to cell death.

Chemotherapy Can be given in addition to another form of

therapy such as radiation or surgery. Drugs may be administered before surgery to

reduce size of tumor. Adjuvant chemotherapy is used after surgery

or radiation therapy to prevent relapse.

Chemotherapy Combination chemotherapy is the use of

more than one class of drug. Administering different classes of chemo

drugs ensures a greater chance of achieving complete cancer cell destruction and achieving remission.

Administration Chemotherapy can be given by mouth,

subcutaneous or intramuscular injections, intravenously, or intrathecally. Oral route used if drug is well absorbed and non

irritating to the GI tract Sub-q or IM: Slow systemic release IV push, piggyback or intravenous infusion

Goals of Chemotherapy Reducing the primary tumor size Destroying cancer cells Preventing metastases and microscopic

spread of the disease

Chemotherapy Drugs Alkylating drug: attack DNA Antimetabolites: interfere with DNA production Antitumor antibiotics: interferes with DNA production Plant alkaloids: prevent cells from dividing Steroid hormones: slow growth of some cancers

Bone Marrow Transplant HSCT: Hematopoictic Stem Cell Transplant:

CHLA has one of the largest program. The option of HSCT depends on the patients

disease, disease status, and general physical condition.

Involves: Umbilical cord blood Parent’s stem cells

Gene Therapy Use of gene therapy in the treatment of

childhood cancer is promising yet complex and still in early phases of clinical application.

Management of Cancer Patient / family education

Begins at time of diagnosis Continues through treatment phases Maintained in post-survival years Support if death of child

Emotional aspects of leukemia http://leukemia.org/pages/413.html

Pain Management Pain caused by disease

Pain with procedures and treatments

Pain associated with side effects of treatment

Pain Management Pharmacologic

Non-Pharmacologic

Sedation or anesthetic medications EMLA cream Conscious sedation

Pain Controlhttp://pedspain.nursing.uiowa.edu/

Immunosuppression and Infection Children with cancer become immune impaired

from a number of causes: Lymphocyte production is altered Splenic dysfunction can prevent maturation of blood

cells and alteration is inflammatory response. Cancer therapy can decrease immunoglobulin

concentrations.

Neutropenia Significant neutropenia can develop during

chemotherapy creating an increased risk of infection in the child with cancer.

Neutropenia occurs when the absolute neutrophil count decreases below 500.

Treatment of Neutropenia Granulocyte colony stimulating factor

decreases the duration of neutropenia by stimulating the proliferation of the progenitor cells of the granulocytes, specifically the neutophils.

G-CSF: 5mcg/kg/day given subcutaneous

Varicella If an immunosuppressed child with no history

of varicella infection or varicella immunization has direct contact with an individual with chickenpox or shingles, varicella zoster immune globulin should be administered.

Acyclovir IV is used in some cases.

Varicella Immunizations Three months after chemotherapy Off prednisone Many will have already had the immunization

as a toddler since it is now a required immunization.

Central Venous Access Devices Two decades ago, CVAD were introduced as

an integral part of the pediatric oncology patient’s treatment plan.

Used to deliver chemotherapy, blood components, antibiotics, fluids, TPN, medications and blood sampling.

CVAD Infection Prevention• Teach family to report signs of catheter

infections: fever, chills, swelling, pain, drainage, or erythema.

• Aseptic technique for dressing changes and heparin flushing.

• Avoid trauma to device• Observe for catheter occlusion

Chemotherapy Side Effect Drugs affect not only the cancer cells but also

healthy cells. Cells most affected are rapidly growing cells

such as hair follicles, reproductive system, bone marrow and gastrointestinal tract.

Management of Side Effects

Malnutrition Occurs in 8 to 32% of the pediatric oncology

population Nutritional goals focus on maintaining normal

growth and development as well as preventing nutritional deficiencies.

Nutrition Interventions Initial nutritional assessment History of child’s eating habits, food allergies,

use of nutritional supplements, base line weight and height measurements.

Enteral feedings at night: preserve intestinal mucosa by keeping it functional

Nausea and Vomiting Most common side effect of cancer treatment. Chemotherapy-associated vomiting is a reflex

controlled by chemoreceptor trigger zone that stimulates the vomiting center in the brain.

Tumor location Radiation therapy Anticipatory nausea

Interventions Antiemetics such as Phenothiazines: (Trilafon),

(Phenergan)and (Thorazine) block dopamine receptors from stimulating the chemoreceptor trigger zones.

Serotonin-receptor antagonist such as Granisetron (Kytril) and Ondansetron (Zofran) are very effective. (>3 years)

Antihistamines: benadryl Administer before chemotherapy

Mucositis Progressive, inflammatory, ulcerative condition of the oral

and gastric mucosa. Occurs due to the interruption of cell renewal process of

the epithelium leading the mucosal atrophy and ulceration Thrombocytopenia or physical trauma may lead to bleeding

and further mucosal damage. Neutropenia and poor dental hygiene predisposes the oral

mucosa to secondary infection.

Interventions Baseline assessment including the oral

cavity, teeth, and gingival mucosa. History of dental exam and use of orthodontic

appliances Meticulous oral care Mouth rinses Monitor hydration status

Constipation Assess normal bowel habits Increase fiber and fluids in diet Stool softeners / colace Physical activity Avoid digital manipulation

Diarrhea Assess for signs of dehydration Record stool patterns IV fluids as needed Low-residue or lactose-free diet Good hand washing

Hair Loss More important in the older child. Most patients will experience hair loss within

10 days of induction chemotherapy Prepare patient for hair loss Males: shave hair Females: short hair style – pick out wig

Psychosocial Support Support groups

Open communication

Daily contact with oncology team

Trusting relationship with nurse

Growth and Development Promote normal G & D Allow decision making Establish daily routines Play therapy Friends School attendance or tutor

Leukemia Most common malignancy

4 in 100,000

Increase in chromosome disorders

High survival rate

Leukemia Unrestricted proliferation of immature WBC’s

in the blood forming tissues of the body.

The cells look different from normal cells and do not function properly.

Prognosis Initial WBC most significant The higher the count the poorer the

outcomes Greater than 100,000 WBC count = poor outcome

Children under 2 years and older than 10 Girls do better than boys

Diagnosis Peripheral blood smear

Bone marrow analysis

Lumbar puncture

Peripheral Blood Smear

Red circles or RBC; large blue are WBC; blue dots are platelets

http://www.fghi.com/careers/html/body_smear.html

Bone Marrow

http://alice.ucdavis.edu/imd/420a/dib/acute/index.htm

Normal Acute Lymphoid Leukemia

Clinical Manifestations Pallor and fever Lethargy Anorexia Weight loss Hemorrhage / petechiae Hepatomegly / splenomegaly

3 Phase Treatment Induction: goal is to achieve remission last

about a month Consolidation: most intensive phase of

chemotherapy lasts 4 to 8 months Maintenance: last two to three years If leukemia cells are detected in bone marrow

process is started all over again.

Induction Therapy Goal of therapy is to achieve remission Leukemia cells are no longer found in the bone

marrow samples, the normal cells return and blood counts become normal.

Drugs used: L-asparaginase, vincristine and a steroid (dexamethasone), for high-risk children a fourth drug (daunorbucin) is often used

Consolidation Phase Several drugs are used in combination to

prevent remaining leukemia cells from developing resistance.

Drugs include: methotrexate and 6-mercaptopurine, vincristine and prednisone

Maintenance If leukemia continues to be in remission

maintenance therapy can be started. Two drugs: vincristine and steroids over a

brief period every 4 to 8 weeks. Duration of total therapy 2 to 3 years.

CNS Therapy CNS prophylaxis is initiated at diagnosis and is used

to reduce the risk for CNS disease. Preventive CNS is based on the premise that the

CNS provides a sanctuary site for leukemic cells that are undetected at diagnosis and reside protected from the action of systemic therapy by the brain blood barrier.

Nursing Interventions Assess for infection Monitor blood values I & O / nutrition Complications of chemotherapy Good hand washing Aseptic technique for blood draws

Leukemia Time Line 1962 cure rate for pediatric cancer is 4 %. 1971 – A combination of chemotherapy and cranial

irradiation proves it can cure at least half of all children with ALL.

1975 – A new combination of chemo drugs helps patients with reccurrence of the disease.

Time Line

1991 Long-term survival rate increases to 73% with intensive induction therapy followed by two years of treatment with eight anti cancer drugs used on a rotating basis.

Time Line 1997 – Bone marrow transplants from

unrelated, genetically matched donors are effective against many childhood leukemia's.

1998 – Study reveals the cure rate for All has increased from 73% to 80%.

CNS Tumors• 2nd most common malignancy• 65% have 5 year survival rate• Most common tumors:

• Astrocytomas 50%• Medulloblastomas 25%• Brain stem gliomas 10%

Clinical Manifestations Classic signs and symptoms are indicative of

increased intracranial pressure. Pressure is due to tumor mass compressing vital

structure, blockage of cerebrospinal fluid flow or tumor associated edema. Gait changes / ataxia Headache with or without vomiting Blurred vision, or diplopia Forceful vomiting upon rising in the morning or

papilledema.

Management Surgery if tumor accessible Chemotherapy Radiation = Reserved for patient older that 2-

years of age Survival rate based on location

Chemotherapy After surgery to prevent tumor from coming

back Shrink tumor that cannot be operated on Shrink tumor so it can be operated on

Chemotherapy Blood brain barrier – natural filter within the body

that allows certain substances through from the blood to the brain tissues.

Drugs used are: temozolamide, procarbazine or lomustine

Methotrexate is injected intra-thecal Implantable wafers: drug is fixed with gel wafer –

drug is slowly released into brain over 2 to 3 weeks

Brain Tumors

Astrocytoma

Large right frontal lobe

neoplasm with small

area of necrosis

Hodgkin's Disease

3rd most common malignancy

15 to 30 years

Three times higher in males

Excellent cure rates

Clinical Manifestations Night sweats

Weight loss

Malaise

Painless, firm nodes

Treatment Radiation to nodes Chemotherapy

Combination therapy for six months Prednisone

Stem cell transplant

Long Term Side Effects Infertility: drugs can damage ovaries or testicles Second cancers: small risk for leukemia in future Heart disease: some drugs can cause heart

problems or radiation to middle of chest Lung damage: pneumonitis from bleomycin

Neuroblastoma Approximately 600 new cases a year. Embryonic tumor Average age of diagnosis is 2 years. Poorest survival rate 50 to 60% have metastases at time of

diagnosis.

Clinical Manifestations Depends on site of tumor

Diagnosis CT scan Bone scan 95% secrete catecholamines in the urine.

Treatment Determined by the stage of disease and age

of child. Children who have localized disease and

complete response to treatment are more likely to achieve a disease free state and long-term survival.

Neuroblastoma

Wilm’s Tumor Most common type of renal tumor in children Approximately 460 new cases each year. Children with hypospadius or cryptorchidism have a

slightly higher incidence. African American and Females at highest risk

Clinical Manifestations Firm non-tender, painless mass in abdomen

Hematuria

Hypertension

Do not palpate the abdomen

CT Scan Wilm’s Tumor

http://www.uhrad.com/pedsarc/peds048.htm

Wilm’s Tumor

Treatment Surgery

Nephrectomy Prevent rupture of capsule Sample for pathology

Chemotherapy and radiation are given based on the stage of the disease.

Osteogenic Sarcoma Malignant tumor of bone

400 new cases each year

Peak incidence is in the second decade of life, when adolescents are gaining vertical height rapidly.

Approximately 20% have metastases at diagnosis

High rate of metastasis to lungs

Diagnosis

Osteosarcoma Tumor

Femur has a largemass involvingthe metaphysis of bone. Tumor has destroyed the cortex.

Anderson’s Pathology

Treatment Limb salvage

Amputation

Chemotherapy

Limb Salvage

http://www.clevelandclinic.org/ortho/tumor/limbSalvageLg.htm

Ewing Sarcoma Tumor of flat bones

Pelvis, chest, vertebrae

Rare in children under 5 years

75% diagnosed by age 20

Ewing Sarcoma

Ewing Sarcoma Tumor

Ewing Sarcoma atdistal end of the tibia.tumor extends intothe soft tissue.

Anderson’s Pathology

Rhabdomyosarcoma Most common soft bone tissue tumor

Head and neck 40% GU 20% Extremities 20% Trunk 15%

Rhabdomyosarcoma

http://www.brisbio.ac.uk/ROADS/subject-listing/face.html

Treatment Surgical removal

Chemo based on tissue biopsy

Radiation

Retinoblastoma Intraocular / Embryonic tumor

1 in 16,000

+ family history

High incidence of malignancies

Retinoblastoma

http://www.djo.harvard.edu/meei/PI/RB/RB.html

Pupil reflex

“Cat Eyes”

Retinoblastoma

http://homepage.idx.com.au/muznsam/

Retinoblastoma

Treatment Surgical enucleation of eye

Genetic counseling

Follow-up care up to 18 Years