Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection

Abstract

Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type 1. Giant neurofibromas are very difficult to manage surgically as they are extensively infiltrative and highly vascularized. These types of lesions require complex preoperative and postoperative management strategies. This case report describes a 22-year-old female with a giant plexiform neurofibroma of the lower back and buttock who underwent pre-operative embolization and intraoperative use of a linear cutting stapler system to assist with haemostasis during the surgical resection. Minimal blood transfusion was required and the patient made a good recovery. This case describes how a multidisciplinary management of these large and challenging lesions is technically feasible and appears to be beneficial in reducing perioperative blood loss and morbidity. Giant neurofibroma is a poorly defined term used to describe a neurofibroma that has grown to a significant but undefined size. Through a literature review, we propose that the term “giant neurofibroma” be used for referring to those neurofibromas weighing 20% or more of the patient's total corporal weight.

Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

“…Some authors reserve this term for tumors weighing more than 20 kg, even though no clear consensus has been reached. These tumors present complex management issues of wound closure, intra-compartmental fluid shifts, and lymphedema [3].…”

“…Clinical management for the PNF requires a multidisciplinary approach [3]. Surgical treatment still remains the mainstream, aiming at improving functional and aesthetic effect [12].…”

“…These entities consist of multiple, twisted masses that grow along the axis of a large nerve, infiltrating and separating normal nerve fascicles [2]. It rarely grows to be larger than 5 cm; however, neurofibromas can undergo continuous enlargement and eventually become giant lesions [3]. Thus, it constitutes a significant source of structural and functional morbidity [4].…”

Preoperative Embolization of a Giant Neurofibroma of the Chest in a Patient with Neurofibromatosis Type II: A Case Report

“…Plexiform neurofibromas can undergo continuous enlargement and eventually become giant lesions (2). If giant plexiform neurofibromas cause symptoms, they require appropriate treatment.…”

Giant Plexiform Neurofibroma of Left Gluteal and Thigh Region: A Case Report

“…Neurofibromatosis type 1 (NF1) is a genetic syndrome which is autosomal dominant caused by mutations in genes encoding for neurofibromin 1 .NF1 is one of the most common human genetic diseases. 1 Incidence estimated to be between 1 and 3000-4000 and equally affect both sexes.…”