Dermatofibrosarcoma protuberans of the vulva treated using mohs micrographic surgery.

dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade sarcoma with a tendency for recurrence. The overall annual incidence is 4.2 per million, or 0.1% of all cancers. It tends to affect young to middle-aged adults and occurs more often in blacks than in whites, with no sex predilection. Dermatofibrosarcoma presents as a flesh-colored, violaceous, or reddish-brown nodule with persistent but indolent growth and is commonly located on the trunk. It rarely metastasizes, but when it does, it is most often to the lungs. Histopathologically, DFSP is a tumor consisting of spindled cells in a storiform pattern. It infiltrates the dermis, the subcutaneous fat, and even the fascia. Tumor cells stain positive for CD34 and negative for factor XIIIa.