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Area of Science:

  • Oncology
  • Pediatric Oncology
  • Dermatopathology

Background:

  • Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing fibrohistiocytic tumor originating in the dermis.
  • Pediatric cases account for 6% of all DFSP diagnoses, with an incidence of 1 per million children.

Purpose of the Study:

  • To review the management and outcomes of pediatric dermatofibrosarcoma protuberans (DFSP) cases.
  • To evaluate the efficacy of surgical excision in achieving recurrence-free survival for pediatric DFSP.

Main Methods:

  • Retrospective review of pediatric DFSP cases managed at a single institution over 23 years.
  • Analysis of patient demographics, treatment modalities, and follow-up data.
  • Surgical excision as the primary treatment approach for all patients.

Main Results:

  • Seventeen pediatric patients (10 male, mean age 9.9 years) were included.
  • Median follow-up was 29 months, with no observed recurrences.
  • Complete surgical excision with clear margins (R0 resection) was achieved in all cases, with some requiring re-excision.

Conclusions:

  • Pediatric DFSP can be effectively managed with surgical excision, leading to excellent outcomes.
  • Multidisciplinary team management involving soft tissue tumor specialists, pathologists, and surgeons is crucial.
  • Achieving R0 resection is key to ensuring recurrence-free survival in pediatric DFSP.