Dermatofibrosarcoma Protuberans: A Review of the Literature

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. MATERIALS AND 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. CONCLUSION: 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.