Chemosensitivity in pediatric dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of fibrohistiocytic spectrum and uncommonly presents in infancy and childhood. The standard treatment is wide-margin surgical excision, but this is difficult to achieve in large tumors and may result in unacceptable cosmetic, structural, and functional consequences. The authors describe a child with recurring DFSP who, after extensive surgery, responded to treatment with vinblastine and oral methotrexate. This is the first reported case in literature of a chemotherapeutic response in pediatric DFSP.