Diagnostic and Management Considerations in Pediatric Dermatofibrosarcoma Protuberans.

ABSTRACT

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumor of dermal origin. Six percent of all cases present in children, with a childhood incidence of 1 per million. METHODS: This is a retrospective review of all cases of pediatric DFSP managed at a single institution over a 23-year period. RESULTS: Seventeen patients (10 male; mean age, 9.9 years) were managed during the study period. The median follow-up was 29 months. All patients had surgical excision. Three patients required further excision to achieve uninvolved final margins. There were no recurrences observed. CONCLUSIONS: Pediatric DFSP should be managed by a soft tissue tumor multidisciplinary team, with experienced pathologists and reconstructive surgeons. Where R0 resections are obtained, patients can experience recurrence-free survival.