Congenital dermatofibrosarcoma protuberans: 30 years of follow-up

Summary Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumor that commonly appears in adult patients. Few cases of DFSP in childhood have been reported. We describe a case of congenital DFSP that had been incompletely excised in childhood, and again at 28 years of age. The 33-year-old woman presented with a recurrence. When comparing the histologic features of the previous specimens excised in 1970 and 30 years later, the similar typical storiform pattern of fibromatous cells was found. The small number of proliferation-marker-positive cells was not altered over the course of 30 years. In line with a few additional reports, we suggest that congenital dermatofibrosarcoma p…