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

Alberto S. Pappo, Bhaskar N. Rao, Alvida Cain, Sara Bodner, Charles B. Pratt

Research output: Contribution to journalArticle

26 Scopus citations

Abstract

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 in proper treatment. Surgical resection (with 3-cm margins) is currently the standard therapy for DFSP.

Original languageEnglish
Pages (from-to)563-568
Number of pages6
JournalPediatric Hematology and Oncology
Volume14
Issue number6
StatePublished - Nov 1 1997

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Keywords

  • Dermatofibrosarcoma protuberans
  • Pediatric
  • Soft tissue sarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology
  • Cancer Research
  • Management of Technology and Innovation

Cite this

Pappo, A. S., Rao, B. N., Cain, A., Bodner, S., & Pratt, C. B. (1997). Dermatofibrosarcoma protuberans: The pediatric experience at St. Jude Children's Research Hospital. Pediatric Hematology and Oncology, 14(6), 563-568.