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.