Dermatofibrosarcoma protuberans in children: Favorable outcomes with wide local excision and narrower margins

Summary: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that is locally aggressive with a high risk of local recurrence if incompletely excised. The National Comprehensive Cancer Network (NCCN) recommends wide local excision (WLE) with 2–4 cm margins or Mohs micrographic surgery (MMS) for adults; however, there are no established pediatric-specific guidelines. As a result, management of DFSP in children is often adapted from adult recommendations. Pediatric patients present unique challenges, because treatment cannot often be performed under local anesthesia, and achieving adequate margins can be difficult due to smaller body size. To address this gap, we conducted a retrospective review of pediatric DFSP patients treated with WLE at a single institution, focusing on surgical margin width and clinical outcomes. Seventeen patients under the age of 20 who were diagnosed with DFSP and treated with WLE between 2004 and 2024 were included. The mean age at diagnosis was 13.2 years; 76.5% were female. The average lesion size was 2.73 cm. 16/17 patients had exact margins recorded. 15/17 (88.2%) were treated with margins ≤2 cm (average 1.76 cm, range 1-3 cm). No patients received adjuvant therapy with imatinib or radiation. At a mean follow-up of 44 months, the cohort demonstrated 0/17 recurrences with 100% disease-specific and overall survival. These findings suggest that the prognosis for children with DFSP is excellent, and that favorable outcomes can be achieved using WLE with margins smaller than adult NCCN guidelines. Jawad Aqeel¹, Claire E. Holtz¹, Kelly Harms¹, Elisabeth A. Pedersen¹ 1. Dermatology, University of Michigan Medical School, Ann Arbor, MI, United States. Clinical Research: Epidemiology and Observational Research