MALIGNANT FIBROUS TUMORS

Dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), and malignant fibrous histiocytoma (MFH) are rare soft tissue sarcomas of intermediate to high-grade malignancy that the dermatologist must evaluate and treat. DFSP is a fibrohistiocytic tumor of intermediate m alignancy characterized by aggressive local growth and propensity to r ecur after surgical excision. AFX is a superficial malignant tumor tha t arises on sun exposed surfaces and is characterized by local aggress ive behavior. MFH is the most aggressive of the fibrohistiocytic tumor s with a high local recurrence rate and significant metastatic rate us ually associated with a poor prognosis. This article reviews the epide miology, pathogenesis, clinical presentation, histology, management, p rognosis, and follow-up of these malignant tumors. Copyright (C) 1998 by W.B. Saunders Company.