Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a relatively unusual, locally aggressive cutaneous tumor of intermediate malignancy, that most frequently occurs with a slight predominance in young adult men on the trunk and proximal extremities. It arises from the dermis and invades deeper subcutaneous tissues (fat, fascia, muscle, bone), but, despite its local invasiveness, it rarely metastasizes (5 % of cases). Currently, the cause of DFSP is unknown. A 54-year-old woman presented with an asymptomatic, red-violaceous ovalar plaque on the left submammary area. The lesion had sharply delineated borders and showed two overlying hanging outgrowths, one smooth and flesh colored and the other reddish and with a cobblestone appearance. Histological and immunohistochemical studies confirmed the diagnosis of DFSP. This unusual presentation of DFSP is presented and discussed.