Dermatofibrosarcoma Protruberance (DFSP) is a rare superficial, intermediate-to-low grade sarcoma, that although metastasizes rarely, is locally very aggressive, with a high recurrence rate. The tumor originates from the dermis and may invade the deeper subcutaneous tissues. Typically, DFSP lesions are firm, with a pink to reddish brown color. Histologically, the lesions appear as densely arranged monomorphous spindle shaped cells, arranged in a storiform pattern. Variants of the typical form include the pigmented form, laden with melanin, (Bednar tumor), as well as the fibrosarcomatous form.
DFSP incidents constitute only less than 0.1% of all malignant neoplasms. It most commonly occurs between the ages of 20 and 50 years. Diagnosis relies on the skin biopsy, since other symptoms are absent. Histologically, the lesions may be confused with benign fibrohistiocytoma, and positive staining with CD34 may assist in such a case. Although metastasis is rare (5% of the cases), most patients with metastatic DFSP die within 2-years. Surgical treatment is the only option. The most commonly applied procedure is wide surgical excision of the lesion, since it is important to have a negative surgical margin. Mohs micrographic surgery has been effectively used in this condition. Chemotherapy is ineffective in combating the condition, although radiotherapy may sometimes be used.