title: Dermatofibrosarcoma protuberans: a review of the literature.
abstract: BACKGROUND
  Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor that is locally aggressive and has traditionally had a high rate of recurrence after surgical intervention.
  
  
  OBJECTIVE
  To review the current literature on DFSP and present its epidemiology, clinical features, histology, immunohistochemisty, tumor biology, prognosis, and treatment options.
  
  
  METHODS
  Literature review using PubMed search for articles related to DFSP.
  
  
  RESULTS
  DFSP is a slow-growing tumor that often presents as a skin-colored plaque on the trunk, although it may arise anywhere on the body. DFSP has a distinctive histologic appearance but can mimic other diseases, and immunhistochemical studies can be helpful in making the diagnosis of DFSP. Most tumors have a translocation between chromosomes 17 and 22. Surgery is the treatment of choice, in the form of Mohs micrographic surgery (MMS) or wide local excision (WLE), although imatinib has emerged as a promising treatment option for advanced disease.
  
  
  CONCLUSIONS
  For DFSP, MMS is associated with a lower rate of recurrence than WLE and is particularly useful for tumors on the head and neck. Imatinib is used in advanced disease and may take on a larger role in the treatment of DFSP in the future.
authors.affiliation: Division of Dermatology, Washington University, Saint Louis, Missouri 63141, USA.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Bogucki
authors.forename: Isaac
authors.initials: I
authors.lastname: Neuhaus
authors.forename: Eva A
authors.initials: EA
authors.lastname: Hurst
mesh_terms: D018223:Dermatofibrosarcoma / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans