Osteosarcoma an overview
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A brief of Osteosarcoma with its clinicopathology, staging and management in short
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Osteosarcoma an overview
- 1. Osteosarcoma Presenter – Dr. Venkatesan A Moderator – Prof. Th. Tomcha Singh
- 2. Introduction • Derived from primitive bone forming mesenchyme & characterized by production of osteoid tissue or immature bone by malignant proliferating spindle cell stroma • 2nd m.c primary malig. tumor of bone • 20% of primary malig. of bone • Incidence 1 – 3 / million per year • 61% - 10 – 20 yrs age • M : F – 1.6 : 1
- 3. Osteosarcoma location & site • Location Metaphysis – 90% Diaphysis – 9% Epiphysis – 1% • Site Femur – 52% Tibia – 20% Humerus – 9%
- 4. Etiology • Radiation exposure • Rx with alkylating agents • In elderly - Paget’s disease - multiple hereditary exostosis - polyostotic fibrous dysplasia • Hereditary disorders - RB - Li – Fraumeni syndrome - Rothmund Thomson syndrome
- 5. Pathology • Gross pathology i. large tumor with destruction of cortex ii. Variable consistency iii. 90% exhibit – codman’s triangle, sunburst app, etc
- 6. Histology • Histologic sub types i. Fibroblastic ii. Chondroblastic iii. Osteoblastic iv. Telengiectactic v. small cell
- 7. Classification A. CENTRAL 1. Primary or idiopathic 2. Secondary B. JUXTA – CORTICAL i. Paraosteal ii. Periosteal iii. High grade surface iv. Dedifferentiated paraosteal
- 8. Clinical features • Pain • Swelling • Occasional pyrexia • Pathologic fracture • Pulmonary mets symptoms
- 9. Work up • History • Physical examn • Routine lab tests • Sr. ALP • X ray • CT scan • MRI • Angiography • Bone scan • Biopsy
- 10. Work up – contd… CT Scan MRI scan
- 11. Mode of spread • Local spread • Hematogenous spread - Approx. 15% dist mets at diagnosis - > 80% pulmonary - Other bony sites • Lymphatic spread - rare
- 12. Staging
- 13. AJCC Staging
- 14. Prognostic factors • Age, localisation, size and initial metastatic disease • most imp. predictor of outcome at diagnosis - - ± metastases. • most reliable prognostic factor - histological response to pre-operative chemotherapy (Huvos)
- 15. Treatment modalities • Surgery • Chemotherapy • Radiotherapy
- 16. Rx – NCCN Guidelines s u r v e i l l a n c e
- 18. Rx – NCCN Guidelines
- 19. SURGERY • Amputation • Limb salvage procedures GUIDELINES FOR LIMB – SPARING RESECTION i. No major neurovascular involvement ii. Wide resection with normal mucsle cuff iii. EN bloc removal of biopsy sites iv. Resection 3-4cm beyond uptake v. Adj. jt resection vi. Adequate motor reconstruction
- 20. Limb salvage surgery Rx by anatomical site • Distal femur • Proximal tibia • Proximal humerus • scapula
- 21. Surgery – contd… • Pelvis & proximal femur - Hemipelvectomy • Surgical Rx of mets • Expandable prothesis in children
- 22. First line therapy (Primary/Neoadjuvant/Adjuvant ) - Cisplatin and doxorubicin/Dactinomycin - MAP (High-dose methotrexate, cisplatin, and doxorubicin) - Doxorubicin, cisplatin, ifosfamide and HDMTX( T-20) - Ifosfamide and etoposide - Ifosfamide, cisplatin and epirubicin Second line therapy (Relapsed or Refractory disease) - Docetaxel and gemcitabine - Cyclophosphamide and etoposide - Ifosfamide and etoposide - Ifosfamide, carboplatin and etoposide - HDMTX, etoposide and ifosfamide - Samarium-153 ethylene diamine tetramethylene phosphonate Current systemic therapy NCCN 2011
- 23. Assessment of histologic response to CT Huvos grading system
- 24. Restaging after Neo adj CT • Clinical evaluation • Plain radiography • Angiography • CT scan • Bone scintigraphy • MRI • PET
- 25. Radiotherapy in Osteosarcoma Indications: - Complete surgical resection not feasible - Inadequate surgical margins - Osteosarcoma of the head and neck with positive or uncertain resection margins - Axial skeleton - Pelvis - Palliation of metastatic bony sarcomas - Adjuvant whole lung irradiation
- 26. RT Treatment Planning and dose Guidelines to optimal RT - Evaluation of extent - Patient immobilization - Planned using CT simulation - reproducible position - Multiple beam-shaping devices - 3D CRT / IMRT Target volume • Large volume fields • Areas to be resected + approx. 2cm margin • Shrinking field technique • Use of extended SSD • Parallel opp. Portal
- 27. RT doses • POST OP RT - 50 – 65 Gy in 25 – 33 # over 5 – 7 wks ( radical) - 40 – 55 Gy in 20 – 28 # over 4 – 5 wks (doubful surgical margins) • PRE OP RT - 35 Gy in 10 # or 25 Gy in 5 # • PALLIATIVE RT - locally advanced 30 – 35 Gy in 10 – 15 # in 2 -3 wks - large bleeding lesions 8 – 10 Gy single #
- 28. RT ….contd • Extracorporeal RT – 250 – 300 Gy • IORT – 45 – 80Gy
- 29. Treatment complications • SURGERY - infection - fracture - non union - joint instability - late osteoarthritis - endoprosthetic loosening - dislocation
- 30. Future outlook - Targeted molecular therapies and newer Novel agents - Minimally invasive approaches - More durable modular oncology prosthesis
- 31. Conclusion Osteosarcoma is a tumor with highly aggressive metastasizing potential Peak incidence between 10 and 19 years of age 80% to 90% of osteosarcomas occur in the long tubular bones 80–90% seemingly localized disease will develop metastases, mostly in the lungs Neo adjuvant and Adjuvant CT along with Limb salvage surgery play a central role .
- 32. Thank you