Please login or create an account. If you do not have access to this content, you will be shown a 30 second preview and licensing options.
- Presentation
Pediatric Dermatology Case Discussion: Managing DFSP and Giant Cell Fibroblastoma with Mohs Surgery
Description
This case discussion focused on a 3-year-old boy with a slowly enlarging mid-chest lesion that was biopsied as a hybrid giant cell fibroblastoma and dermatofibrosarcoma protuberans (DFSP), with a characteristic COL1A1-PDGFB fusion. After re-excision, the deep margin remained positive, prompting a review of next-step options including observation, repeat wide excision, Mohs surgery, imatinib, or radiation. The speaker reviewed pediatric DFSP variants, emphasizing that these tumors can be difficult to diagnose clinically, can have significant subclinical extension, and that recurrence is problematic. The talk highlighted that negative margins are critical and that Mohs offers complete circumferential peripheral and deep margin assessment, but in children it can be logistically challenging because it often requires general anesthesia, coordination with pediatric surgery/anesthesia, and prolonged operative time. Discussion participants noted the need for multidisciplinary care, better access to pediatric Mohs expertise, and the possible role of slow Mohs in situations where standard frozen-section Mohs is impractical. The family ultimately preferred Mohs to minimize the chance of recurrence, underscoring the importance of individualized risk-benefit discussions in pediatric patients.
View moreConclusions
- Pediatric DFSP is often deceptively subtle and can be difficult to recognize clinically, so early biopsy is important.
- Because pediatric DFSP may extend far beyond visible borders, wide margins based only on inspection can miss disease.
- The central management goal is complete negative-margin excision, since recurrence risk rises sharply when margins remain positive.
- Mohs micrographic surgery or other complete margin-assessment techniques generally provide lower recurrence rates than conventional bread-loafed wide local excision.
- However, pediatric Mohs is logistically challenging because many children require general anesthesia and some lesions require prolonged or multi-stage operations.
- In children, the choice between Mohs and wide local excision must balance oncologic certainty against anesthesia burden and surgical complexity.
- Slow Mohs may be a practical alternative in some pediatric settings when same-day Mohs infrastructure is difficult to coordinate.
- Pediatric DFSP usually has an excellent prognosis and rarely metastasizes, but recurrence can be locally destructive and is best avoided with definitive initial treatment.
- Multidisciplinary coordination among dermatology, Mohs surgery, plastic surgery, anesthesia, and sometimes neurosurgery or ENT is essential for complex pediatric cases.
- The presentation argues that pediatric DFSP can warrant Mohs in selected cases, especially when recurrence risk, infiltrative growth, or anatomy make margin control critical.
- Rust DJ, Kwinta BD, Geskin LJ, Samie FH, Remotti F, Yoon SS. Surgical management of dermatofibrosarcoma protuberans. J Surg Oncol. 2023 Jul;128(1):87-96. doi: 10.1002/jso.27258. Epub 2023 Mar 31. PMID: 36999599.#10.1002/jso.27258
- NCCN Guidelines Version 2.2026 for Dermatofibrosarcoma Protuberans.
- Dermatofibrosarcoma Protuberans in Children: Favorable Outcomes Using Wide Local Excision.#10.1111/pde.70087
- A Comparison of Mohs Micrographic Surgery and Wide Local Excision for Treatment of Dermatofibrosarcoma Protuberans With Long-Term Follow-up: The Mayo Clinic Experience.#10.1097/dss.0000000000000910
- Suzuki, Kim, and Barlow. Cutis. 2024.
- Journal of the American Academy of Dermatology systematic review of Mohs micrographic surgery in children.
- Pediatric Dermatology article on Mohs surgery for cutaneous malignancies in pediatric age groups.#10.1016/s0738-081x(01)00169-9