A Case of Congenital Dermatofibrosarcoma Protuberans with a Histologic Feature of Bednar Tumor / 대한피부과학회지

ABSTRACT

Dermatofibrosarcoma protuberans(DFSP) is a cutaneous mesenchymal neoplasm originating in the dermis, which is diagnosed most in adulthood, with a range in age from birth to the ninth decade. Congenital DFSP is extremely rare and the diagnosis is difficult because of its resemblance to vascular birth mark of skin atrophy. We describe a case of congenital DFSP in a 19-year-old female on the left forearm, which was present as a vascular-appearing macule from birth with recent nodular change. Interestingly, the histopatholgy revealed Bednar tumor, a pigmented DFSP and it has not been reported in congenital case yet. The patient was treated with a wide and deep excision with surgical margin of 4cm and STSG, and no recurrence was noted for one year.