[Dermatofibrosarcoma protuberans of the face. A case report]

Report of a dermatofibrosarcoma protuberans (DFSP) of the face with a follow-up of more than 40 years in a 63-year-old women. A total of 64 operations and a radiotherapy were performed to treat the recurrences, which increased in number in the last 9 years. Defects were mostly closed primarily, with free grafts or local flaps. Two metastases, probably of yatrogenic origin, were found at the skin of the clavicula and the breast one year after a Bakamjian flap at the former site of the drains. Histology revealed mature fibroblasts growing in bundles, cart-wheel or storiform with destructive infiltration of the dermis. The DFSP is mainly a locally malignant tumor, growing slowly and painless as a flesh-coloured multinodular mass localised in the dermis. Local recurrence occurs in more than 50% of the cases, whereas metastases are very rare.