Dermatofibrosarcoma protuberans: the pediatric experience at St. Jude Children's Research Hospital

The clinical characteristics and outcome of children with dermatofibrosarcoma protuberans (DFSP) treated at a single institution over a 34-year period were reviewed. A retrospective chart review identified five children with dermatofibrosarcoma protuberans. The diagnosis was confirmed in all cases by one of the authors. Age; sex; clinical group; tumor invasiveness and size, nodal status, and metastases (TNM stage); therapy; and outcome were retrieved from the medical records of these patients. The median age at diagnosis was 12.5 years. There were four boys, and all tumors arose in the extremities or trunk. All children underwent at least two surgical procedures to achieve negative or surgically microscopic positive margins. In addition, one patient received adjuvant radiotherapy for microscopically positive margins. All patients survive free of disease 1 month to 16.5 years after diagnosis. Dermatofibrosarcoma protuberans is rare among children and adolescents. Recognition of this pathologic entity is important for its proper treatment. Surgical resection (with 3-cm margins) is currently the standard therapy for DFSP.