Download - Osteosarcoma (1)
- 1.Osteo = bone/osteoid tissue Sarcoma = malignant tumour of connective tissue 02/04/12 Dr. Pruthviraj Nistane Deptt. Of Orthopaedics Govt. Medical College and Rajindra Hospital, Patiala
2. Overview
- Definition
- Epidemiology
- Pathogenesis
- Skeletal distribution
- Clinical presentation
- Evaluation
- Classification
- Investigations
- Treatment
- Prognosis
3. What is osteosarcoma ?
- Highly malignant tumor of mesenchymal origin.
- Spindle shaped cells that produce osteoid.
- 2 ndmost common primary malignant bone tumor after MM.
4. Epidemiology
- Incidence 1 to 3 per million per year
- Any age
- But 75%in 12-25yrs of age
- Almost equal in both sexes, slightly more in males .
5. Epidemiology
- Associatedsyndromes
- Hereditary form of retinoblastoma
- Li-Fraumeni syndrome (p53)
- Rothmund-Thomson syndrome (8q24)
6. Pathogenesis
- Unknown
- Modal incidence correlates with rapid bone growth
- Radiation >2000 rads
- Chemicals chlorantherene, AAF, Be compounds
7. Gross pathology
- Arise from multipotent mesenchymal cells
- Mixture of osteoid, fibrous, cartilaginous, necrotic,
- hemorrhagic, cystic areas
- Destruction of cortex
8. Gross pathology
- Metaphyseal, Central.
- Extension into medullary cavity and subperiosteal extension.
- Restricted bu periosteun and epiphyseal plate, but eventually crosses it
- Reactive periosteal
- new bone formation
- Metastasis lungs
9. Microscopic appearance
- Stroma - Malignant connective tissue with anaplastic spindle cells
- Matrix of osteoid/fibrous/cartilagenous tissue
10. Classification
- PRIMARY or SECONDARY
- PRIMARY OSTEOSARCOMAS are
- Conventional /classicosteosarcoma (highgrade, intra medullar y)
- Low-grade intramedullary osteosarcoma
- Parosteal osteosarcoma
- Periosteal osteosarcoma
- High-grade surface osteosarcoma
- Telangiectatic osteosarcoma, and
- Small cell osteosarcoma.
11. Classification
- SECONDARY OSTEOSARCOMAS
- Osteosarcomas occurring at the site of another diseaseprocess.
- more common in >50 years of age
- The most common causes are
- Paget disease
- Previous radiation treatment
- Other associated conditions are
- Fibrous dysplasia
- Bone infarcts
- Osteochondromas
- Chronic osteomyelitis
- Dedifferentiated chondrosarcomas
- Osteogenesis imperfecta
12. Classic High Grade Osteosarcoma
- These aggressive, high-grade tumors begin in an intramedullary location, but may break through the cortex and form a soft-tissue mass.
- The histologic hallmark -malignant osteoblastic spindle cells producing osteoid,presence of woven bone with malignant appearing stromal cells
- subtypes-
- osteoblastic,
- chondroblastic and
- fibroblastic
13. Skeletal distribution
- Distal femur
- Proximal tibia
- Proximal humerus
- (sites of rapid bone growth)
- others
- Metaphyseal(89%)>diaphyseal(10%)>epiphyseal(1%)
14. Clinical Presentation
- Pain progresssive pain
- due to microinfarction
- night pain in 25 %
- Swelling-Palpable mass is noted in up to 1/3
- of patients at the first visit
- Fever, malaise or other constitutional symptoms
- are not typical of osteosarcoma
15. Evaluation
- Suspected diagnosis by history and physical examination
- Supported by investigations
16. Plain X-ray(Most valuable)
- sclerotic
- Lytic
Mixed (most common) 17. Plain X-ray
- Lesions are usually permeative
- Associated with destruction of the cancellous and cortical elements of the bone
- Ossification within the soft tissue component, if tumour has broken through cortex
- Intra medullary
- Borders are ill defined
18. Plain X-ray
- Periosteal reactionmay appear as the characteristic Codman triangle.
- Extension of the tumor through the periosteum may result in a so-called sunburst or hair on end appearance.
19. Other investigations
- MRI
- CT
- Angiogram
- Bone scan
- Laboratory studies
- Biopsy
20. MRI
- best to detect extent into bone and soft tissues
21. CT
- Not of much use
- CT chest to detect lung metastasis
22. Angiogram
- Determine vascularity of the tumour
- Detect vascular displacement
- Relationship of vessels to the tumour
23. Bone scan
- A bone scan should be obtained
- to look for skeletal metastases
- or multi focal disease
- Thallium scan - Monitor effects of chemotherapy
- Detect local recurrence of tumor
24. laboratory studies
- Full blood count, ESR, CRP.
- LDH (elevated level is associated with poorprognosis)
- ALP (highly osteogenic)
- Platelet count
- Electrolyte levels
- Liver function tests
- Renal function tests
- Urinalysis
25. Biopsy
- to conform the diagnosis.
- Types
- Fine needle aspiration
- Core needle biopsy
- Openincisional biopsy
26. Enneking staging system
- The staging system is typically depicted as follows
- Stage I: Low grade tumors
- I-A intra compartmental
- I-B extra compartmental
- Stage II: High grade tumors
- II-A intra compartmental
- II-B extra compartmental
- Stage III: Any tumors with evidence ofmetastasis
27. Differential Dx
- Giant Cell Tumor
- Chondrosarcoma
- Fibrosarcoma
- Aneursymal Bone Cyst
- Ewings sarcoma
- Osteoblastoma
- Metastasis
- Lymphoma
- Osteomyelitis
- Chondroblastoma
- Post traumatic callus
- Other variants
28. Surface osteosarcoma
- Parosteal
- Periosteal
- High grade surface osteosarcoma
29. Parosteal
- 5% of osteosarcomas
- Posterior metaphysis of
- distal femur
- Arises from surface,invade
- medullary cavity in late stages
- Tends to encircle bone
- Low grade,Slow growing
- Large ossified mass in centre
30. Periosteal
- Arises from surface of diaphysis
- Most commonly femur and tibia
- Characterized by bony spicule formation
- perpendicular to shaft
- Strands of osteoid producing spindle cells
- radiating between lobules of cartilage
- Sunburst
- Low grade
31. High grade surface
- Very rare
- Age group 20-30s
- Appearance as parosteal but histology high grade and medullary involvement more common.
32. Telangiectatic Osteosarcoma
- Aggressive
- Presents with pathological fracture
- 5% of all osteosarcomas
- arises within the diaphysis
- Radiology
- Often entirely osteolytic
- Bone and cortex destruction
- Periosteal reaction
- Codman's triangles
- Pathology
- Gross appearance is a multi-cystic similar to an aneurysmal bone cyst.
- Microscopically it has large blood filled spaces and thin septation. Within the septa there is scanty osteoid production by the pleomorphic malignant cells
33. Prognostic Factors
- Extent of the disease
-
- Pts with pulmonary, non pulmonry (bone) or skip metastasis have poor prognosis
- Grade of the tumor
-
- High grade tumor have poor prognosis
- Size of the primary lesion
-
- Large size tumors have worse prognosis then small size tumors
- Skeletal location
-
- proximal tumors do worse than distal tumors.
- Secondary osteosarcoma:Poor prognosis
34. Treatment
- Current standard of care
- Radiological staging
- Biopsy to confirm diagnosis
- Preoperative chemotherapy
- Repeat radiological staging (access chemo response, finalize surgical treatment plan)
- Surgical resection with wide margin
- Reconstruction using one of many
- techniques
- Post op chemo based on preop response
35. Chemotherapy
- Chemotherapy given preoperatively - Neoadjuvant
- Given postoperatively -Adjuvant
- Advantages ofneoadjuvantchemotherapy -
- regression of the primary tumor, making a successful limbsalvage operation easier.
- may decrease the spread of tumor cells at the time of surgery
- Effectively treating micrometastases at the earliest time possible.
- It avoid tumor progression, which may occurduring any delaybefore surgery.
- Given for about 3-4 weeks before definitive procedure
36. Chemotherapy
- The drugs used most often to treat osteosarcoma are:
- Methotrexate with leucovorin (folinic acid)
- Doxorubicin (Adriamycin)
- Cisplatin or carboplatin
- Etoposide
- Ifosfamide
- Cyclophosphamide
- Actinomycin D (dactinomycin)
- Bleomycin
37. Surgery
- Themain goal of surgery is to safely and completely remove the tumor.
- Historically amputation.
- Over the past few years -limb-sparing procedures have become the standard, mainly due to advances in chemotherapy and sophisticated imaging techniques
- Limb salvage procedures now can provide rates of local control and long-term survival equal to amputation.
38. Surgery choice
- Surgical procedures fall into three basic categories:
- Amputation
- Limb salvage
- Rotationplasty
39. Decision ???
- If the tumor can be removed safely while retaining a viable extremity, a limb sparing procedure may be appropriate.
- If major nerves or blood vessels are involved, or if complete tumor removal results in significant loss of function, amputation may be a better choice.
- Patients age, desired level of function, cosmetic preference and long-term prognosis must also be considered.
40. Amputation
- Amputation involves removal of the limb with a safe margin between the end of
- the retained portion and the
- tumor
- It should not be viewed as a
- failure of treatment, but rather
- as the first step towards patients return to a more comfortableand productive life.
41. Amputation
- Indication
-
- 1. Grossly displaced pathologic fracture
-
- 2. Encasement of neurovascular bundle
-
- 3. Tumor that enlarges during preop chemo andis adjacent to neurovascular bundle
-
- 4. Palliative measure in metastatic disease
-
- 5. If the tumor has caused massive necrosis,fungation, infection, or vascular compromise.
42. Limb salvage surgery
- Removing the tumor with a normal cuff of tissue surrounding it while preserving vascular and nerve supply to the extremity.
43.
- The skeletal defect must be reconstructed by
- Endoprosthesis (most common)
- replacing the removed bone with
- a metal implant
- Allograft (cadaveric) bone
- Vascularized bone acquired from the patient
- Allograft-prosthetic composite constructions
44. Rotationplasty
- compromise between amputation and limb salvage
- most commonly used for osteosarcomas of the distal femur in skeletally immature patients
- It is a procedure where the neurovascular structures and distal aspect of the limb (leg) are retained, and re-attached to the proximal portion after the tumor has been removed.
45.
- For functional purposes, the distal segment is turned 180 degrees so that the ankle joint functions as a knee joint, thus converting an above-knee to a below-knee amputation in order for prosthetic use to be maximized
46. Radiotherapy
- Radiation therapy has no major role in osteosarcoma
- Radiation therapy may be useful in some cases where the tumor cannot be completely removed by surgery. E.g. in pelvic bones or in the bones of the face.
- Megavoltage (upto 6000-8000 rads)
47. Follow up and Prognosis
- Signs of recurrence, metastasis and treatment related complications
- Physical examination,radiographs of the primary site, serial chest imaging,bone scans and laboratory examinations
- 50 % cases with high grade osteosarcoma have some type of relapse in 5 months
- If recurrence is detected, additional surgery(radical amputation)and chemotherapy may be warranted.
- 5 year survival rate is 5% - 23%
48.
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