[Juvenile dermatofibrosarcoma protuberans].

Dermatofibrosarcoma protuberans is observed in younger or middle-aged patients after the transformation from the initial stage of a chronic fibrous plaque into a rapidly growing, sometimes painful or bleeding tumor. First manifestation in early childhood is considered in about 10 per cent of the reported patients. In the case that we observed the neoplasma was present at birth and increased to a multinodular tumor between three and four years of age. Repeated local recurrences in the scar were observed after the surgical removal. The clinical behavior of the tumor in childhood is comparable to those of adults. Because of its malignant potential extended early excision should be undertaken despite of the risk of widespread scarring.