Dermatofibrosarcoma Protuberans in Children: Favorable Outcomes Using Wide Local Excision

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).

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.

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%.

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.