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Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report

BACKGROUND: Giant neurofibromas in patients with neurofibromatosis type 1 involve multiple regions and are often difficult to surgically extirpate. However, surgical intervention is the most effective means for improving quality of life. The case reported herein is unique in that it involves a giant...

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Autores principales: Shen, Xiang-qian, Shen, Hui, Wu, Shou-cheng, Lv, Ying, Lu, Hui, Lin, Xiang-jin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4848791/
https://www.ncbi.nlm.nih.gov/pubmed/27122017
http://dx.doi.org/10.1186/s12957-016-0880-y
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author Shen, Xiang-qian
Shen, Hui
Wu, Shou-cheng
Lv, Ying
Lu, Hui
Lin, Xiang-jin
author_facet Shen, Xiang-qian
Shen, Hui
Wu, Shou-cheng
Lv, Ying
Lu, Hui
Lin, Xiang-jin
author_sort Shen, Xiang-qian
collection PubMed
description BACKGROUND: Giant neurofibromas in patients with neurofibromatosis type 1 involve multiple regions and are often difficult to surgically extirpate. However, surgical intervention is the most effective means for improving quality of life. The case reported herein is unique in that it involves a giant neurofibroma, involving the patient’s peritoneal and pelvic cavities, retroperitoneal space, and buttock, which was causing compressive displacement of abdominal and pelvic organs. A challenging surgical intervention was required to accomplish near-total resection to relieve organ compression while preserving visceral and genitoanal function. CASE PRESENTATION: The case reported is of a patient presenting with a solitary giant retroperitoneal neurofibroma that threatened to obliterate both peritoneal and pelvic cavities and protruded conspicuously into the right gluteal region. The enormous dumbbell-shaped mass was surgically removed in three parts. Postoperative pathology studies confirmed a diagnosis of neurofibroma. Follow-up computed tomography images taken three months postoperatively revealed residual tumor in the perianal region. The patient’s quality of life had measurably improved on follow-up at eight months. CONCLUSIONS: Surgical intervention in such extraordinary circumstances of a giant neurofibroma causing compressive displacement of critical organs reduces tumor burden, restores appearance and function of patient’s body and internal organs, and improves the patient’s quality of life.
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spelling pubmed-48487912016-04-29 Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report Shen, Xiang-qian Shen, Hui Wu, Shou-cheng Lv, Ying Lu, Hui Lin, Xiang-jin World J Surg Oncol Case Report BACKGROUND: Giant neurofibromas in patients with neurofibromatosis type 1 involve multiple regions and are often difficult to surgically extirpate. However, surgical intervention is the most effective means for improving quality of life. The case reported herein is unique in that it involves a giant neurofibroma, involving the patient’s peritoneal and pelvic cavities, retroperitoneal space, and buttock, which was causing compressive displacement of abdominal and pelvic organs. A challenging surgical intervention was required to accomplish near-total resection to relieve organ compression while preserving visceral and genitoanal function. CASE PRESENTATION: The case reported is of a patient presenting with a solitary giant retroperitoneal neurofibroma that threatened to obliterate both peritoneal and pelvic cavities and protruded conspicuously into the right gluteal region. The enormous dumbbell-shaped mass was surgically removed in three parts. Postoperative pathology studies confirmed a diagnosis of neurofibroma. Follow-up computed tomography images taken three months postoperatively revealed residual tumor in the perianal region. The patient’s quality of life had measurably improved on follow-up at eight months. CONCLUSIONS: Surgical intervention in such extraordinary circumstances of a giant neurofibroma causing compressive displacement of critical organs reduces tumor burden, restores appearance and function of patient’s body and internal organs, and improves the patient’s quality of life. BioMed Central 2016-04-27 /pmc/articles/PMC4848791/ /pubmed/27122017 http://dx.doi.org/10.1186/s12957-016-0880-y Text en © Shen et al. 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Shen, Xiang-qian
Shen, Hui
Wu, Shou-cheng
Lv, Ying
Lu, Hui
Lin, Xiang-jin
Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report
title Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report
title_full Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report
title_fullStr Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report
title_full_unstemmed Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report
title_short Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report
title_sort surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4848791/
https://www.ncbi.nlm.nih.gov/pubmed/27122017
http://dx.doi.org/10.1186/s12957-016-0880-y
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