Dermatofibrosarcoma protuberans of the upper lip: an overview and a case report.

Dermatofibrosarcoma protuberans is a slow-growing but locally aggressive malignant neoplasm of the skin. The preferential locations are on the trunk and proximal extremities but it can occur in the head and neck region in about 15% of the cases. This is a report of such a case and a review of the literature of head and neck cases attempting to analyse the profile of patients and surgical prognosis factors. The recurrence rate is important (20% to 55%) and dependent on the free tissue margins. Those margins are at least 3 cm in all directions; this is difficult to assess in the head and neck region because of both functional and cosmetic aspects. Distant metastases are rare but related to a long recurrence history. This behaviour gives the impression that prognosis is poorer for dermatofibrosarcoma in the head and neck than within other locations.