DERMATOFIBROSSARCOMA PROTUBERANS EM IDADE PEDIÁTRICA

Dermatofibrosarcoma protuberans is a rare fibrohistiocytic tumor with frequent locoregional recurrences and a low metastatic ability. Only 6% of dermatofibrosarcoma protuberans are diagnosed in children. The gold standard treatment is the surgical excision and the lower recurrence rates are observed with wide surgical margins (2,5cm) or with margin control by Mohs micrographic surgery. The authors report the case of a female 8-year-old child with a dermatofibrosarcoma protuberans of the abdominal wall. The tumor was excised with wide margins with a good outcome after a 12-month follow-up. The typical features of this tumor in children and the main differential diagnoses were pointed out.