Bone and soft tissue tumor
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Bone and soft tissue tumor
Rare but significant in young adults in India, including sarcomas and benign tumors.
1. Bone Tumors
Common Malignant Types
Tumor
Typical Age Group
Common Site
Osteosarcoma
Adolescents/ young adults
Metaphysis of long bones (e.g., distal femur, proximal tibia)
Ewing Sarcoma
Children/ young adults
Pelvis, femur, ribs
Chondrosarcoma
Adults (>40 years)
Pelvis, femur, shoulder
Multiple Myeloma
Older adults
Axial skeleton (spine, ribs, pelvis)
Risk Factors
- Genetic conditions (e.g., Li-Fraumeni, RB mutations, Paget's disease of bone)
- Prior radiation exposure
- Chronic osteomyelitis (rarely leads to sarcoma)
Treatment
-
Osteosarcoma/Ewing:
- Neoadjuvant chemotherapy → surgery (limb-sparing or amputation) → adjuvant chemo
- Radiation (e.g., Ewing) if unresectable or post-op residual disease
-
Chondrosarcoma:
- Mainly surgical resection; chemo/radiation less effective
-
Multiple Myeloma:
- Systemic therapy (e.g., bortezomib, lenalidomide, steroids) + RT for pain control
2. Soft Tissue Sarcomas (STS)
Common Types
- Rhabdomyosarcoma (children)
- Liposarcoma, leiomyosarcoma, undifferentiated pleomorphic sarcoma (UPS) (adults)
- Synovial sarcoma, angiosarcoma, MPNST (neurogenic origin)
Common Sites
- Extremities (most common), retroperitoneum, trunk, head & neck
Risk Factors
- Genetic syndromes (e.g., Li-Fraumeni, NF1)
- Previous radiation therapy
- Chronic lymphedema (e.g., Stewart-Treves syndrome)
Treatment:
- Surgery: Wide local excision with negative margins is primary treatment
Radiation Therapy:
- Preoperative RT: 50 Gy in 25 fractions
- Postoperative RT: 60–66 Gy in 30–33 fractions
Chemotherapy:
- Role varies; used more in high-grade, large, or metastatic tumors
- Rhabdomyosarcoma: chemo is integral (VAC regimen – vincristine, actinomycin D, cyclophosphamide)
Role of Radiation Therapy
Indication
RT Use
Bone Tumors (e.g., Ewing, MM)
Pre/post-op or definitive if unresectable (45–55 Gy)
Soft Tissue Sarcomas
Pre-op RT (50 Gy) or post-op RT (60–66 Gy) for margin control
Palliative RT
Pain relief, control of bleeding or compressive symptoms
Key Points
- Multimodal approach (surgery, RT, chemo) is often needed
- Limb preservation is now standard over amputation when feasible
- RT is crucial in local control, especially for STS and Ewing sarcoma