Surgical management of a giant plexiform neurofibroma of the lower extremity.

Abstract

The authors review their experience with the management of a giant 49-kg (108 lb) neurofibroma of the lower extremity in a 37-year-old male with NF1. The patient presented with right thigh pain, paresthesias, increasing edema, and accelerated growth of the mass. The patient was taken to the operating room, where approximately 39 kg (86 lb) of tumor and 10 L (22 lb) of interstitial fluid was removed. The postoperative course was complicated by cellulitis, delayed wound closure, and recurrent lymphedema. Complications were managed with antibiotics, ultrasound-guided drainage, surgical revision of closure, compressive dressings, passive drainage, and vacuum-assisted wound closure. Giant neurofibromas similar to this tumor require complex intraoperative and postoperative management strategies. Surgical closure is best managed with local flaps, and postoperative lymphedema is best managed with passive drainage and compressive dressings.