Dermatofibrosarcoma Protuberans in Children: Favorable Outcomes Using Wide Local Excision

Background Dermatofibrosarcoma protuberans (DFSP) is a rare and locally aggressive cutaneous sarcoma. Surgical excision remains first‐line therapy, including for pediatric patients. However, given the rarity of DFSP, specific treatment recommendations for children have not been well defined. To inform pediatric‐specific management, we analyzed outcomes in a retrospective cohort of pediatric DFSP patients treated with wide local excision (WLE). Methods A single‐center retrospective review of clinical records from 2004 through 2024 was conducted evaluating patients < 20 years of age who were diagnosed with DFSP and underwent treatment with WLE. Patients were divided into two groups based on treatment with relatively narrow (< 2 cm) or standard margins (≥ 2 cm). Descriptive analyses were performed. Results A total of 17 pediatric DFSP patients underwent WLE. The mean surgical margin was 1.76 ± 0.54 cm, and 14 of 16 evaluable patients (87.5%) were treated with margins of 2 cm or less. Complete excision after one surgery was achieved in 76.5% of patients. No patients experienced local or distant recurrence, and none required adjuvant therapy. Interestingly, 64.7% (11/17) had documentation describing the lesion being first noted at or around the time of birth. For patients with over 60 months of follow‐up, the 5‐year recurrence‐free survival, disease‐specific survival, and overall survival were all 100%. Conclusions When appropriately selected and performed, WLE with a 1–2 cm initial margin, followed by re‐excision when needed to achieve clear margins, does not compromise survival or risk of recurrence and may be considered an effective treatment option in pediatric DFSP.