Multidisciplinary management of a giant plexiform neurofibroma by double sequential preoperative embolization and surgical resection.

David Barastegui,Roberto Vélez,Sergi Barrera-Ochoa,Cleofe Romagosa,Mercedes Pérez-Lafuente,Manuel Pérez

doi:10.1155/2013/987623

David Barastegui, Roberto Vélez + Show 4 more

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https://doi.org/10.1155/2013/987623

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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.

Highlights

Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic syndrome caused by mutations in genes coding for neurofibromin
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

Summary

Introduction

Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic syndrome caused by mutations in genes coding for neurofibromin. The incidence of NF1 has been estimated to be between 1 and 3000–4000 [1, 2] and affects male and female subjects in all races This mutation predisposes patients to the development of multiple neurofibromas [3]. There are a number of case reports and series found in the literature discussing giant neurofibromas [7,8,9,10,11,12,13,14,15,16]. We report a case of a giant PNF of the lower back and buttock. It was managed with double sequential intravascular embolization followed by surgical resection, demonstrating that this technique is technically feasible and should be considered for these lesions. Through a literature review, we propose a new definition of giant neurofibroma

Case Report

Discussion

Findings

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