ABSTRACT

Fibrosarcoma (or fibroblastic sarcoma) is a malignant tumor that originates in the connective fibrous tissue at the ends of bones, and then spreads to other surrounding soft tissues. Fibrosarcoma is a tumor of mesenchymal cell origin containing immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform/cartwheel pattern. Fibrosarcoma may arise de novo or emerge as a secondary tumor from a preexisting benign lesion. Risk factors for fibrosarcoma include previous radiotherapy, bone infarct, surgically treated fracture or joint reconstruction, preexisting benign lesions (e.g., enchondroma, bizarre parosteal osteochondromatous proliferation, chronic osteomyelitis, giant cell tumor, or fibrous dysplasia), known malignant lesion (e.g., low-grade chondrosarcoma), Paget disease, and inherited genetic conditions (e.g., Gardner syndrome, Li-Fraumeni syndrome, retinoblastoma, and multiple neurofibromas). Treatment options for fibrosarcoma consist of radical excision with an adequate margin, radiation if residual tumor or positive margins, and chemotherapy if high grade.