Dermatofibrosarcoma Protuberans: an Update and Review

Dermatofibrosarcoma protuberans (DFSP) is a rare, infiltrative, spindle cell tumor with a propensity for deep invasion. Definitive treatment is with surgical excision, either Mohs micrographic surgery (MMS) or wide local excision (WLE). Metastasis is rare, but local recurrence is common. Imatinib mesylate is approved for the treatment of unresectable DFSP, recurrent DFSP that cannot be re-resected, and metastatic DFSP. Radiation therapy is sometimes used to treat unresectable DFSP or as an adjuvant treatment modality after surgical excision. MRI is not routinely used but is a useful imaging tool in atypical, recurrent, or difficult cases to better delineate tumor depth or involvement of critical structures. Close clinical follow-up is recommended every 6 to 12 months after treatment given the high rates of local recurrence.