Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a very rare tumor. It is a rare neoplasm of the dermis layer of the skin, and is classified as a sarcoma. There is only about 1 case per million per year. DFSP is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma. In many respects, the disease behaves as a benign tumor, but in 2-5% of cases it can metastasize, so it should be considered to have malignant potential. It occurs most often in adults in their thirties; it has been described congenitally, in children, and the elderly. It accounts for approximately 2-6% of soft tissue sarcoma cancers.