Diagnostic and Management Considerations in Pediatric Dermatofibrosarcoma Protuberans

Ann Plast Surg. 2022 Mar 1;88(3):319-322. doi: 10.1097/SAP.0000000000002970.

Abstract

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumor of dermal origin. Six percent of all cases present in children, with a childhood incidence of 1 per million.

Methods: This is a retrospective review of all cases of pediatric DFSP managed at a single institution over a 23-year period.

Results: Seventeen patients (10 male; mean age, 9.9 years) were managed during the study period. The median follow-up was 29 months. All patients had surgical excision. Three patients required further excision to achieve uninvolved final margins. There were no recurrences observed.

Conclusions: Pediatric DFSP should be managed by a soft tissue tumor multidisciplinary team, with experienced pathologists and reconstructive surgeons. Where R0 resections are obtained, patients can experience recurrence-free survival.

MeSH terms

  • Child
  • Dermatofibrosarcoma* / diagnosis
  • Dermatofibrosarcoma* / pathology
  • Dermatofibrosarcoma* / surgery
  • Follow-Up Studies
  • Humans
  • Male
  • Margins of Excision
  • Neoplasm Recurrence, Local / diagnosis
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / surgery
  • Retrospective Studies
  • Skin Neoplasms* / diagnosis
  • Skin Neoplasms* / pathology
  • Skin Neoplasms* / surgery