Related Experiment Video
Updated: Jan 9, 2026
Lateral Molar Approach-Driven Transoral Endoscopic Procedure for Benign Infratemporal Fossa Tumor Resection
Published on: August 15, 2025
Dermatofibrosarcoma Protuberans in Children: Favorable Outcomes Using Wide Local Excision
Jawad Aqeel1, Claire E Holtz2, Grace A Osborne3
1Department of Dermatology, Division of Cutaneous Surgery and Oncology, University of Michigan Medical School, Ann Arbor, Michigan, USA.
Insights
Wide local excision (WLE) is effective for pediatric dermatofibrosarcoma protuberans (DFSP). This treatment, even with narrow margins, shows excellent survival and low recurrence rates in children.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Dermatopathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, aggressive skin cancer.
- Pediatric DFSP lacks specific treatment guidelines.
- Wide local excision (WLE) is the primary treatment for DFSP.
Purpose of the Study:
- To evaluate outcomes of WLE in pediatric DFSP patients.
- To inform pediatric-specific DFSP management strategies.
- To analyze recurrence and survival rates after WLE.
Main Methods:
- Retrospective review of 17 pediatric DFSP patients treated with WLE (2004-2024).
- Comparison of outcomes based on surgical margin width (<2 cm vs. ≥2 cm).
- Descriptive analysis of patient data and treatment results.
Main Results:
- Complete excision achieved in 76.5% of patients with margins ≤2 cm.
- No local or distant recurrence observed; no adjuvant therapy needed.
- 100% 5-year recurrence-free, disease-specific, and overall survival rates.
Conclusions:
- WLE with 1-2 cm margins is effective for pediatric DFSP.
- Re-excision for clear margins does not compromise outcomes.
- WLE is a viable treatment option for pediatric DFSP.
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.
