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 malignancy characterized by aggressive local growth and propensity to recur after surgical excision. AFX is a superficial malignant tumor that arises on sun-exposed surfaces and is characterized by local aggressive behavior. MFH is the most aggressive of the fibrohistiocytic tumors with a high local recurrence rate and significant metastatic rate usually associated with a poor prognosis. This article reviews the epidemiology, pathogenesis, clinical presentation, histology, management, prognosis, and follow-up of these malignant tumors.
Department of Dermatology, The University of Rochester, NY 14642, USA.
F J
FJ
Stadler
G A
GA
Scott
M D
MD
Brown
D017024:Chemotherapy, Adjuvant; D018223:Dermatofibrosarcoma / Q000175:diagnosis* / Q000453:epidemiology / Q000556:secondary / Q000628:therapy*; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D015580:Mohs Surgery / Q000379:methods; D009367:Neoplasm Staging; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012307:Risk Factors; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000145:classification*