Dermatofibrosarcoma protuberans presenting in infancy and childhood

Dermatofibrosarcoma protuberans (DFSP) is a not uncommon low-grade cutaneous sarcoma of uncertain histogenesis, which typically arises in early to middle adult life. Traditionally, it is regarded as extremely uncommon in infants and children, and this diagnosis may therefore easily be overlooked in young patients. Eight such cases (representing 5.9% of the available DFSPs on file) are presented of which two were congenital. Age range at presentation was 14 months to 12 years. Five arose on the trunk. Most had originally been mistaken for unclassified sarcoma, a fibromatosis, or diffuse neurofibroma. The histologic features were entirely comparable to the more usual adult cases except that all had a plaque-like, rather than nodular, growth pattern. Short-term follow up has revealed no recurrences. DFSP is not so rare in childhood and warrants wider recognition in order to ensure appropriate treatment.