LE DERMATOFIBROSARCOME DE DARIER ET FERRAND, UNE TUMEUR CUTANEE PARTICULIERE

Introduction: The dermatofibrosarcoma protuberans (DFS) is a fibrous tumor of the skin, with slow growth, and very high-risk of local recurrence, but with low metastatic potential. Patients and methods: Starting from a retrospective study spread out over 5 years (2002 - 2007), we analyzed the epidemiologic and clinical characteristics, the time of diagnosis, the type of therapeutic and the evolution of 32 patients presenting the dermatofibrosarcoma tumors, followed to the national center of burned and plastic surgery. Among the 32 patients, 10 were presented initially to the service for a tumoral recurrence The DFS reached the young adult preferentially. The diagnostic time is on average 4 years. The trunk is the preferential localization (60%) Results: The 32 patients were treated by surgical excision with a margin of 5cm on the surface, an excision in-depth of an healthy anatomical barrier. The cover was made after anatomopathologic confirmation of the carcinological character of the excision, and it’s often made with skin graft The evolution was marked by occurred of a tumoral recurrence at 8 patients and the results were considered to be satisfactory on the esthetic and functional level. Discussion: On the epidemiologic and clinical level, the data are traditional and similar with the series of the literature; (the age of beginning, the sex, the clinical aspect, size and localization of the tumor) The Broad initial surgical excision is the treatment of reference. Recent studies are in favor of a reduction of the margins to 3 cm but the results are still to evaluate. Conclusion: The DFS of Darier and Ferrand is a tumor whose forecast and the evolutionary risk are mainly related to the time diagnosis and the quality of the first excision. The late diagnosis, makes difficult the excision and reconstructive surgery. The improvement of the forecast require the sensitizing and the information of the general doctor for the early diagnosis and the correct orientation of these patients towards specialized centres.