Dermatofibrosarcome de Darier et Ferrand congénital : un cas pédiatrique

Summary Introduction Dermatofibrosarcoma show an extremely aggressive tendency to invade surrounding tissue. It was first described in 1924. It usually occurs in young men. This type of tumor is exceptional in childhood. The authors report a case of congenital dermatofibrosarcoma diagnosed in a child. Report of case A two-year old female patient presented with a tumor of the vertex scalp since her birth. Biopsy revealed a dermatofibrosarcoma. The tumor was removed surgically with 3 cm margins. The primary reconstruction was performed using a double temporoparietal flap (H). There was no recurrence at five years of follow-up. Discussion Congenital dermatofibrosarcoma is very rare. Only twenty cases have been reported.