Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant tumor of the skin, the diagnosis of which can be difficult not only clinically but also histologically. The tumor has a low metastatic potential, but tends to recur locally if removal is incomplete.

We reviewed the clinical and histologic features of 19 cases of DFSP, seen at the Department of Dermatology (14 cases) and Department of Surgery (5 cases) at the Hull Royal Infirmary between 1975 and 1992.

There were only 19 cases of DFSP diagnosed from a population of 500,000 over a period of 17 years. None of the cases were diagnosed clinically prior to histologic diagnosis. The highest incidence was in the 4th decade of age. The most frequent site was on the head and neck. None of the lesions metastasized but there were a total of eight incidents of local recurrence in five patients. Histologically all the cases showed presence of storiform-pattern spindle-shaped cells and new collagen formation.

Dermatofibrosarcoma protuberans is a soft tissue tumor that is often not recognized clinically due to its rarity and variable appearance. Local recurrence is frequent. A wide surgical excision is the treatment of choice.