Dermatofibrosarcoma Protuberans: What Is This?

Surg Clin North Am. 2022 Aug;102(4):657-665. doi: 10.1016/j.suc.2022.05.004.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive dermal-based sarcoma. Metastatic potential is extremely low, primarily in the setting of fibrosarcomatous transformation. DFSP is characterized by a t(17;22) (q22;q13) translocation that results in active PDGFB signaling. Surgical resection with negative margins (typically including the underlying fascia) is the potentially curative treatment. Delayed wound closure should be considered for cases requiring extensive resection or tissue rearrangement. Tyrosine kinase inhibitors, such as imatinib, have shown response rates of 50% to 60% in patients with locally advanced or metastatic disease. Radiation can be useful for residual or recurrent diseases.

Keywords: DFSP; Dermatofibrosarcoma protuberans; Surgical considerations.

Publication types

  • Review

MeSH terms

  • Dermatofibrosarcoma* / diagnosis
  • Dermatofibrosarcoma* / genetics
  • Dermatofibrosarcoma* / therapy
  • Humans
  • Imatinib Mesylate
  • Margins of Excision
  • Skin Neoplasms* / genetics
  • Skin Neoplasms* / pathology
  • Skin Neoplasms* / therapy

Substances

  • Imatinib Mesylate