Management of a large abdominal dermatofibrosarcoma protuberans requiring a life-threatening excision: A case report

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant tumor of the dermis and subcutaneous tissue, characterized by local aggressiveness and a high recurrence rate. The gold standard treatment is wide excision with negative margins, sometimes using Mohs surgery, with careful planning for reconstruction. In challenging cases, neoadjuvant imatinib therapy and adjuvant radiotherapy may help optimize outcomes. We report a case of extensive abdominal DFSP requiring a multidisciplinary approach after resection exposed the liver and the last three right ribs.

Neoadjuvant imatinib was administered to reduce tumor size, followed by radical resection and immediate reconstruction using prosthetic mesh and three pedicled flaps: a deep inferior epigastric perforator flap, an anterior intercostal artery perforator flap, and a pedicled latissimus dorsi flap. The reconstruction was performed as a single-stage procedure. Postoperative ischemia due to hematoma required conversion of the latissimus dorsi flap into a free flap and remobilization of the remaining flap. A split-thickness skin graft was applied on postoperative day ten. Histology confirmed negative margins. At six months, the patient showed complete healing, no recurrence, and a satisfactory reconstructive outcome.

In such extensive DFSP cases, immediate flap reconstruction helps prevent complications related to exposed bone or viscera and preserves functional and aesthetic outcomes. It does not hinder oncologic follow-up and may reduce morbidity.

This case highlights the surgical and reconstructive challenges of large DFSPs and the vital role of a plastic surgery team in planning tailored, multidisciplinary management within an oncodermatology center.