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

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...

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Main Authors: Roberto Vélez, Sergi Barrera-Ochoa, David Barastegui, Mercedes Pérez-Lafuente, Cleofe Romagosa, Manuel Pérez
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2013/987623
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author Roberto Vélez
Sergi Barrera-Ochoa
David Barastegui
Mercedes Pérez-Lafuente
Cleofe Romagosa
Manuel Pérez
author_facet Roberto Vélez
Sergi Barrera-Ochoa
David Barastegui
Mercedes Pérez-Lafuente
Cleofe Romagosa
Manuel Pérez
author_sort Roberto Vélez
collection DOAJ
description 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.
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institution Kabale University
issn 2090-6668
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language English
publishDate 2013-01-01
publisher Wiley
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series Case Reports in Neurological Medicine
spelling doaj-art-1a870458c9964676ac41765bc7c1f4882025-02-03T06:11:33ZengWileyCase Reports in Neurological Medicine2090-66682090-66762013-01-01201310.1155/2013/987623987623Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical ResectionRoberto Vélez0Sergi Barrera-Ochoa1David Barastegui2Mercedes Pérez-Lafuente3Cleofe Romagosa4Manuel Pérez5Orthopaedic Oncology Unit, Orthopaedic Surgery and Traumatology Department, Hospital Universitari Vall d'Hebron, Pg Vall d'Hebron 129-139, 08035 Barcelona, SpainOrthopaedic Surgery and Traumatology Department, Hospital Universitari Vall d'Hebron, Pg Vall d'Hebron 129-139, 08035 Barcelona, SpainOrthopaedic Surgery and Traumatology Department, Hospital Universitari Vall d'Hebron, Pg Vall d'Hebron 129-139, 08035 Barcelona, SpainInterventional Radiology Service, Radiology Department, Hospital Universitari Vall d'Hebron, Pg Vall d'Hebron 129-139, 08035 Barcelona, SpainPathology Department, Hospital Universitari Vall d'Hebron, Pg Vall d'Hebron 129-139, 08035 Barcelona, SpainOrthopaedic Oncology Unit, Orthopaedic Surgery and Traumatology Department, Hospital Universitari Vall d'Hebron, Pg Vall d'Hebron 129-139, 08035 Barcelona, SpainPlexiform 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.http://dx.doi.org/10.1155/2013/987623
spellingShingle Roberto Vélez
Sergi Barrera-Ochoa
David Barastegui
Mercedes Pérez-Lafuente
Cleofe Romagosa
Manuel Pérez
Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection
Case Reports in Neurological Medicine
title Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection
title_full Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection
title_fullStr Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection
title_full_unstemmed Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection
title_short Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection
title_sort multidisciplinary management of a giant plexiform neurofibroma by double sequential preoperative embolization and surgical resection
url http://dx.doi.org/10.1155/2013/987623
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