Abstract
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. 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 3cm margins. The primary reconstruction was performed using a double temporoparietal flap (H). There was no recurrence at five years of follow-up. Congenital dermatofibrosarcoma is very rare. Only twenty cases have been reported.
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