Surgery Combined With Embolization in the Treatment of Plexiform Neurofibroma: A Case Report and Literature Review

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal dominant multisystem genodermatosis resulting from a mutation on chromosome 17q11.2, characterized by diverse clinical expression with involvement of the skin, nervous system, bones, eyes and other systems. Neurofibromas are tumors located along nerve bundles; when they become large, they are called plexiform neurofibromas. Complications of neurofibromas are rare, but include malignant transformation and potentially life-threatening hemorrhages. A comprehensive perioperative management, including arterial embolization of feeding vessels, is required in order to perform a safe surgical procedure and to reduce potential surgical complications. We report a case of a large plexiform neurofibroma on the right flank, treated in a two-stage approach: first, embolization with polyvinyl alcohol particles of the inferior epigastric, lumbar and superior gluteal arteries on the right and, second, 24 hours after embolization, exeresis of the lesion, which weighed 2.5 kg. Two years after surgery, the patient was asymptomatic, without tumor recurrence and with an important improvement in self-esteem and quality of life