Hybrid resection of a giant thigh plexiform neurofibroma

• Massive plexiform neurofibromatosis is a rare expression of von Recklinghausen’s disease or Type 1 neurofibromatosis.
• Tumor progression is a serious problem for patients with a plexiform neurofibroma due to the debilitating nature of the disease to a patient’s function and potential for malignant transformation.
• Treatment has two goals, to restore function and to restore cutaneous appearance. This demands a multidisciplinary approach with preoperative embolization central to minimize peri-operative hemorrhage.

Presentation of caseAuthors present a case of a 55 year old patient with Type 1 neurofibromatosis (NF1) and a very large right thigh plexiform neurofibroma. The patient had increasing difficulty with mobilization due to this enlarging bulky mass. Preoperative embolization reduced the bleeding risk at surgery allowing successful gross resection of the mass by a multidisciplinary surgical team. Limb function was restored to normal.DiscussionMassive plexiform neurofibromatosis is a rare expression of von Recklinghausen's disease or NF1. These large masses result in severe disfigurement and significant functional disability. They are extremely vascular and there is potential for malignant transformation.ConclusionThese massive tumors require complex preoperative, intraoperative and postoperative management strategies with involvement from a multidisplinary team. We discuss the challenges of surgical intervention and to discuss the current literature.