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A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case

Mesenteric desmoid-type fibromatosis (DTF) is a rare benign yet aggressive neoplasm that has an unpredictable biological behavior ranging from spontaneous regression to extensive local infiltration and has a high tendency for recurrence. The presenting symptoms are usually nonspecific and mostly rel...

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Autores principales: Rabai, Nimah A, Alrababah, Arqam, Batayha, Shaima A, Ba-shammakh, Saleh A, Sayaheen, Mohammad O
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10611482/
https://www.ncbi.nlm.nih.gov/pubmed/37900485
http://dx.doi.org/10.7759/cureus.46087
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author Rabai, Nimah A
Alrababah, Arqam
Batayha, Shaima A
Ba-shammakh, Saleh A
Sayaheen, Mohammad O
author_facet Rabai, Nimah A
Alrababah, Arqam
Batayha, Shaima A
Ba-shammakh, Saleh A
Sayaheen, Mohammad O
author_sort Rabai, Nimah A
collection PubMed
description Mesenteric desmoid-type fibromatosis (DTF) is a rare benign yet aggressive neoplasm that has an unpredictable biological behavior ranging from spontaneous regression to extensive local infiltration and has a high tendency for recurrence. The presenting symptoms are usually nonspecific and mostly related to the large size of the tumor compressing adjacent organs. Imaging studies can be suggestive of the diagnosis, but confirmation is based on histopathological and immunohistochemical examination. The lack of knowledge on the etiology and pathogenetic behavior of this tumor leads to therapeutic and prognostic challenges. Future genetic studies may help in advancing our understanding of this neoplasm and in formulating the proper management and follow-up plan. Here we present a case of a 14-year-old female who presented to the emergency room complaining of diffuse abdominal pain and distention. A computed tomography (CT) scan showed a large mass occupying most of the abdominal cavity and compressing adjacent organs. Exploratory laparotomy with resection and anastomosis was performed, and the histopathological and immunohistochemical examination of the resected mass was consistent with mesenteric DTF.
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spelling pubmed-106114822023-10-28 A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case Rabai, Nimah A Alrababah, Arqam Batayha, Shaima A Ba-shammakh, Saleh A Sayaheen, Mohammad O Cureus Pathology Mesenteric desmoid-type fibromatosis (DTF) is a rare benign yet aggressive neoplasm that has an unpredictable biological behavior ranging from spontaneous regression to extensive local infiltration and has a high tendency for recurrence. The presenting symptoms are usually nonspecific and mostly related to the large size of the tumor compressing adjacent organs. Imaging studies can be suggestive of the diagnosis, but confirmation is based on histopathological and immunohistochemical examination. The lack of knowledge on the etiology and pathogenetic behavior of this tumor leads to therapeutic and prognostic challenges. Future genetic studies may help in advancing our understanding of this neoplasm and in formulating the proper management and follow-up plan. Here we present a case of a 14-year-old female who presented to the emergency room complaining of diffuse abdominal pain and distention. A computed tomography (CT) scan showed a large mass occupying most of the abdominal cavity and compressing adjacent organs. Exploratory laparotomy with resection and anastomosis was performed, and the histopathological and immunohistochemical examination of the resected mass was consistent with mesenteric DTF. Cureus 2023-09-27 /pmc/articles/PMC10611482/ /pubmed/37900485 http://dx.doi.org/10.7759/cureus.46087 Text en Copyright © 2023, Rabai et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Pathology
Rabai, Nimah A
Alrababah, Arqam
Batayha, Shaima A
Ba-shammakh, Saleh A
Sayaheen, Mohammad O
A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case
title A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case
title_full A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case
title_fullStr A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case
title_full_unstemmed A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case
title_short A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case
title_sort sporadic giant mesenteric desmoid-type fibromatosis in a teenage female: a report of a rare case
topic Pathology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10611482/
https://www.ncbi.nlm.nih.gov/pubmed/37900485
http://dx.doi.org/10.7759/cureus.46087
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