DERMATOFIBROSARCOMA PROTUBERANS - THE PEDIATRIC EXPERIENCE AT ST.-JUDE-CHILDRENS-RESEARCH-HOSPITAL

The clinical characteristics and outcome of children with dermatofibro sarcoma protuberans (DFSP) treated at a single institution over a 34-y ear period were recieved. A retrospective chart review identified five children with dermatofibrosarcoma protuberans. The diagnosis was conf irmed in all cases by one of the authors. Age; sex; clinical group; tu mor invasiveness and size, nodal status, and metastases (TNM stage); t herapy; and outcome were retrieved from the medical records of these p atients. The median age at diagnosis was 12.5 years. There were four b oys, and all tumors arose in the extremities or trunk. All children un derwent at least two surgical Procedures to achieve negative or surgic ally microscopic positive margins. In addition, one patient recieved a djuvant radiotherapy for microscopically positive margins. All patient s survive free of disease 1 month to 16.5 years after diagnosis. Derma tofibrosarcoma protuberans is rare among children and adolescents. Rec ognition of this pathologic entity is important for its proper treatme nt. Surgical resection (with 3-cm margins) is currently the standard t herapy for DFSP.