A brief of Osteosarcoma with its clinicopathology, staging and management in short

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
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17. Rx –NCCN guidelines
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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