Radiation Oncology/Sarcoma/Fibrosarcoma
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Fibrosarcoma
Benign Fibrous Histologies
edit- Nodular fasciitis: rapid growth over several weeks, pain, tenderness. Plump mature fibroblasts. Self-limiting process; recurrence uncommon after excision
- Fibroma: nonspecific term. Dense fibrous nodule. Simple excision
- Elastofibroma: rare, slow-growing benign tumor. Typically lower portion of the scapula. Repetitive manual tasks. Considered reactive lesions. Swollen eosinophilic collagen and elastic fibers. Simple excision
- Superficial fibromatosis: arise from fascia or aponeuroses, small and slow growing
- Palmar fibromatosis (Dupuytren's contracture) most common
- Penile fibromatosis (Peyronie's disease) relatively rare, though more common in men who have palmar fibromatosis
- Deep fibromatosis: clinically diverse, deep-seated fibrous neoplasms
- For more detail, please see the Desmoid tumor page
Fibrosarcoma Epidemiology
edit- 2-6% of primary malignant bone lesions
- Arise most commonly in tubular long bones, usually femur or tibia
- Most patients are 30-70; equal men and women
- Predisposing factors (~30% cases) include Paget's, fibrous dysplasia, osteomyelitis, bone infarcts, bone cysts, benign giant cell tumors, desmoplastic fibromas, or previous RT treatment
Natural History
edit- Locally aggressive
- Metastatic potential related to grade
- High grade similar to osteosarcoma: 5-year OS ~25%
- Low grade: 5-year (and 10-year) OS ~50%. Mets rate 5-15%
- Survival: 5-year 34%, 10-year 28%, 20-year 25%
- Children have relatively milder prognosis
Diagnosis
edit- Lytic with no reactive sclerosis
- High grade lesions show cortical destruction and periosteal reaction; sometimes with soft-tissue mass
- Radiographically very similar to osteosarcoma
Staging
edit- Please see the bone staging page
Treatment Overview
edit- Aggressive surgical resection
- Not considered highly radiosensitive, but RT recommended for inoperable tumors, residual disease, and palliation
- Perez (4th ed) recommends doses 66-70Gy with shrinking-field technique