Malignant fibrous tumors

Semin Cutan Med Surg. 1998 Jun;17(2):141-52. doi: 10.1016/s1085-5629(98)80007-2.


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.

Publication types

  • Review

MeSH terms

  • Chemotherapy, Adjuvant
  • Dermatofibrosarcoma / diagnosis*
  • Dermatofibrosarcoma / epidemiology
  • Dermatofibrosarcoma / secondary
  • Dermatofibrosarcoma / therapy*
  • Diagnosis, Differential
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Mohs Surgery / methods
  • Neoplasm Staging
  • Prognosis
  • Radiotherapy, Adjuvant
  • Risk Factors
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / therapy*
  • Soft Tissue Neoplasms / classification*