%0 Journal Article
%T Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection
%A Roberto V¨¦lez
%A Sergi Barrera-Ochoa
%A David Barastegui
%A Mercedes P¨¦rez-Lafuente
%A Cleofe Romagosa
%A Manuel P¨¦rez
%J Case Reports in Neurological Medicine
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/987623
%X 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. 1. Introduction Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic syndrome caused by mutations in genes coding for neurofibromin. NF1 is one of the most common human genetic diseases. The incidence of NF1 has been estimated to be between 1 and 3000¨C4000 [1, 2] and affects male and female subjects equally in all races. This mutation predisposes patients to the development of multiple neurofibromas [3]. Neurofibromas are common, representing approximately 5% of all benign soft-tissue tumors in large surgical series. Three types of neurofibromas are classically described: localized, diffuse, and plexiform [4¨C6]. Plexiform neurofibromas (PNFs) rarely grow to be larger than 5£¿cm; however, neurofibromas can undergo continuous enlargement and eventually become giant lesions. Early childhood, puberty, and childbearing age are considered to be the periods of greatest risk for disease progression. Furthermore, PNFs have a potential for transformation into highly malignant peripheral nerve sheath tumors, which occur in approximately 5% of patients [7]. Giant neurofibroma is a poorly defined term used to describe a neurofibroma that has grown to a significant but undefined size. There are a number of case reports and series found in the literature discussing giant neurofibromas [7¨C16]. PNFs are difficult to manage surgically as they are
%U http://www.hindawi.com/journals/crinm/2013/987623/