Life Threatening Complications following Excision of Giant Neurofibroma

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.

A neurofibroma‐like tumor in a giant congenital nevus

“…Of course, more extensive blood loss could have been expected if the tumor had indeed been a neurofi broma. In such cases, preoperative ligation of the supplying vessels with the aid of computed tomography angiography could be performed [ 7 ] .…”

Ein neurofibromartiger Tumor in einem kongenitalen Riesennävus

“…Natürlich hätte der Blutverlust auch größer sein können, wenn der Tumor tatsächlich ein Neurofibrom gewesen wäre. In solchen Fällen könnte eine präoperative Ligatur der versorgenden Gefäße mit Hilfe der CT‐Angiographie durchgeführt werden .…”