Dermatofibrosarcoma protuberans and the Bednar tumor: Treatment in the pediatric population

Dermatofibrosarcoma protuberans (DFSP) is a low-intermediate-grade cutaneou s sarcoma that has a marked propensity for local recurrence after excision. The Bednar variant of this tumor is even less common and is distinguished histologically by the dispersal of melanin containing cells in an otherwise typical DFSP. Both are considered to be tumors of the third and forth deca des of life, but both DFSP and the Bednar Variant have been described in ch ildren. Until this report of a congenital Bednar tumor, only the DFSP has a lso been described in the neonate. The histopathology and surgical manageme nt of DFSP and Bednar tumors are outlined with emphasis on reported experie nce in the pediatric population. The surgical management of these lesions i n children is based on numerous series in adults and the limited pediatric experience. The recommended treatment is wide excision with 3-cm margins of visibly uninvolved tissue and inclusion of superficial fascia. J Pediatr S urg 33:1811-1814. Copyright (C) 1998 by W.B. Saunders Company.