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Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature

BACKGROUND: Desmoid fibromatosis (DF) is a rare clonal proliferation of fibroblasts and myofibroblasts. It develops in the connective tissues and does not metastasize but may infiltrate adjacent structures. Because of the rarity of these tumors and the unpredictable natural history of the disease, w...

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Autores principales: Zhao, Jiming, Cheng, Fajuan, Yao, Zhigang, Zheng, Bin, Niu, Zhihong, He, Wei
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9037953/
https://www.ncbi.nlm.nih.gov/pubmed/35478728
http://dx.doi.org/10.3389/fsurg.2022.851164
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author Zhao, Jiming
Cheng, Fajuan
Yao, Zhigang
Zheng, Bin
Niu, Zhihong
He, Wei
author_facet Zhao, Jiming
Cheng, Fajuan
Yao, Zhigang
Zheng, Bin
Niu, Zhihong
He, Wei
author_sort Zhao, Jiming
collection PubMed
description BACKGROUND: Desmoid fibromatosis (DF) is a rare clonal proliferation of fibroblasts and myofibroblasts. It develops in the connective tissues and does not metastasize but may infiltrate adjacent structures. Because of the rarity of these tumors and the unpredictable natural history of the disease, well-defined and precise guidelines of the optimal treatment for DF have not been formulated. CASE PRESENTATION: Here, we present a giant abdominal DF that invaded the right spermatic cord and iliac vessels. The lesion was excised with external iliac artery dissection; however, the vein was sacrificed. The abdominal wall defect was then repaired with a polypropylene mesh. The lesional cells are positive for β-catenin. CONCLUSIONS: In the past decades, there has been a change in the treatment of DF. The “wait and see” policy has been considered initially in most cases. Surgical intervention remains a valid option for symptomatic lesions. The optimal regimes of the tumor should not take the risk of making the patient more symptomatic than the lesion itself.
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spelling pubmed-90379532022-04-26 Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature Zhao, Jiming Cheng, Fajuan Yao, Zhigang Zheng, Bin Niu, Zhihong He, Wei Front Surg Surgery BACKGROUND: Desmoid fibromatosis (DF) is a rare clonal proliferation of fibroblasts and myofibroblasts. It develops in the connective tissues and does not metastasize but may infiltrate adjacent structures. Because of the rarity of these tumors and the unpredictable natural history of the disease, well-defined and precise guidelines of the optimal treatment for DF have not been formulated. CASE PRESENTATION: Here, we present a giant abdominal DF that invaded the right spermatic cord and iliac vessels. The lesion was excised with external iliac artery dissection; however, the vein was sacrificed. The abdominal wall defect was then repaired with a polypropylene mesh. The lesional cells are positive for β-catenin. CONCLUSIONS: In the past decades, there has been a change in the treatment of DF. The “wait and see” policy has been considered initially in most cases. Surgical intervention remains a valid option for symptomatic lesions. The optimal regimes of the tumor should not take the risk of making the patient more symptomatic than the lesion itself. Frontiers Media S.A. 2022-04-11 /pmc/articles/PMC9037953/ /pubmed/35478728 http://dx.doi.org/10.3389/fsurg.2022.851164 Text en Copyright © 2022 Zhao, Cheng, Yao, Zheng, Niu and He. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Surgery
Zhao, Jiming
Cheng, Fajuan
Yao, Zhigang
Zheng, Bin
Niu, Zhihong
He, Wei
Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature
title Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature
title_full Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature
title_fullStr Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature
title_full_unstemmed Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature
title_short Surgical Management of a Giant Desmoid Fibromatosis of Abdominal Wall With Vessels Invasion in a Young Man: A Case Report and Review of the Literature
title_sort surgical management of a giant desmoid fibromatosis of abdominal wall with vessels invasion in a young man: a case report and review of the literature
topic Surgery
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9037953/
https://www.ncbi.nlm.nih.gov/pubmed/35478728
http://dx.doi.org/10.3389/fsurg.2022.851164
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