Staged Resection and Reconstruction of a 60-kg Giant Plexiform Neurofibroma Involving the Posterior Trunk and Thigh

Source: PubMed Central Open Access, NCBI / U.S. National Library of Medicine

Summary: Neurofibromatosis type 1 is a genetic disease that causes the development of numerous neurofibromas, with large plexiform forms being rare but associated with significant reconstructive challenges. Giant plexiform neurofibromas involving the posterior trunk and lower limbs, especially those exceeding 30 kg, are extremely rare. We report the case of a 41-year-old man with neurofibromatosis type 1 who had a massive plexiform neurofibroma weighing 60 kg. The tumor originated from the posterior trunk and extended to the gluteal and posterior thigh regions, exceeding his own body weight. Branches of the right profunda femoris artery, bilateral iliolumbar arteries, and the bilateral internal iliac arteries were embolized preoperatively in an attempt to control intraoperative bleeding. The tumor was resected in 2 stages and was reconstructed sequentially with negative pressure wound therapy, split-thickness skin grafts, and regional flaps. The postoperative period was complicated by repeated infections and multiple flap failures. However, the final wound closure and stable soft-tissue condition were eventually achieved through a staged multidisciplinary approach. This case highlights the importance of staged resection and individualized reconstructive techniques for the surgical treatment of exceptionally large plexiform neurofibromas, particularly in high-risk patients.