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  • Presentation

Pediatric Dermatofibrosarcoma Protuberans: Mohs Surgery, Margins, and Management Challenges

Description

The speaker discussed a challenging pediatric dermatofibrosarcoma protuberans (DFSP) case in a 3-year-old boy whose lesion on the mid-chest was biopsied as a hybrid giant cell fibroblastoma/DFSP with a COL1A1-PDGFB fusion. After an initial re-excision left a positive deep margin, the team debated next steps, including observation, another wide excision, Mohs surgery, imatinib, or radiation. The talk emphasized that pediatric DFSP can be difficult to diagnose because of its varied clinical appearances, may extend far beyond visible borders, and recurrence can be disastrous. The speaker reviewed that negative margins are crucial, Mohs offers complete circumferential peripheral and deep margin assessment and tends to lower recurrence compared with standard wide excision, but in children it brings major logistical and anesthetic challenges, especially when general anesthesia is required and multidisciplinary coordination is needed. A “slow-Mohs” approach was presented as a possible alternative when same-day frozen section Mohs is impractical. Overall, the message was that pediatric Mohs has an important role in selected cases, especially DFSP and other rare aggressive tumors, but treatment must be individualized based on tumor extent, location, and the burdens of repeated or prolonged anesthesia.

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Conclusions

  • Pediatric DFSP can be difficult to recognize clinically because it often mimics benign lesions, so early biopsy is important.
  • The tumor can extend far beyond its visible borders, making complete excision more extensive than expected.
  • Achieving negative margins is the central goal, because recurrence is strongly associated with positive or incomplete margins.
  • Mohs surgery offers the most reliable margin control for DFSP by examining the entire peripheral and deep margin rather than only a sampled fraction.
  • Compared with wide local excision, Mohs appears to have lower recurrence rates in the broader DFSP literature.
  • In children, the best operation must balance oncologic control against the practical burdens of anesthesia, logistics, and reconstruction.
  • When prolonged anesthesia is a concern, slow Mohs or staged approaches may be a useful alternative in selected cases.
  • Pediatric DFSP generally has an excellent prognosis, but recurrence can become catastrophic and much harder to manage after inadequate initial surgery.
  • Multidisciplinary coordination is often necessary for pediatric Mohs because children may need plastics, ENT, neurosurgery, or other specialists during resection and reconstruction.
  • Because pediatric-specific evidence is limited, management should be individualized based on tumor features, location, and the family’s ability to tolerate the procedure.
  • 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
  • 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
  • Systematic review of Mohs micrographic surgery in children: Identifying challenges and practical considerations for successful application.#10.1016/j.jaad.2020.09.052
  • Mohs micrographic surgery for cutaneous malignancies: A focus review of its indications in pediatric age groups.#10.1111/pde.13460
  • NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans
  • Suzuki, Kim, and Barlow. Cutis, 2024.