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A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report

Desmoid type fibromatosis (DTF) is a rare benign tumor of connective tissue origin. While these tumors are typically not malignant, they can exhibit aggressive growth patterns causing mass effect on surrounding organs. These tumors typically present in the extremities and abdominal wall, rarely occu...

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Autores principales: Sioda, Natasha A., Wakim, Andre A., Wong, Tina, Patel, Shyamal, Coan, Kathryn, Row, David
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7493805/
https://www.ncbi.nlm.nih.gov/pubmed/33117845
http://dx.doi.org/10.3389/fsurg.2020.00060
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author Sioda, Natasha A.
Wakim, Andre A.
Wong, Tina
Patel, Shyamal
Coan, Kathryn
Row, David
author_facet Sioda, Natasha A.
Wakim, Andre A.
Wong, Tina
Patel, Shyamal
Coan, Kathryn
Row, David
author_sort Sioda, Natasha A.
collection PubMed
description Desmoid type fibromatosis (DTF) is a rare benign tumor of connective tissue origin. While these tumors are typically not malignant, they can exhibit aggressive growth patterns causing mass effect on surrounding organs. These tumors typically present in the extremities and abdominal wall, rarely occurring in the mesentery, and abdominal organs. Due to the rarity of this tumor and the variable size and origin, it is difficult to provide exact prognosis, recurrence, and treatment efficacy regarding desmoid tumors arising from the ileocolic mesentery. We present a case of a young male with a sporadic 31 × 25 × 12 cm DTF arising from the ileocolic mesentery that was complicated by mass effect on bowel and intra-abdominal organs requiring surgical intervention. On presentation, the patient exhibited weight gain and abdominal pressure. Abdominal distension without tenderness on palpation was noted on physical examination. The tumor biopsy confirmed the diagnosis of DTF. No evidence of familial adenomatous polyposis or Gardner syndrome was identified. The tumor was surgically excised and intimately associated with the bowel requiring ileocolonic resection with primary anastomosis. At 3-months follow up, surveillance MRI showed no residual or recurrent lesion. A multi-disciplinary approach to this patient's diagnosis and treatment allowed for an accurate diagnosis, efficient treatment, and follow up plan.
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spelling pubmed-74938052020-10-27 A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report Sioda, Natasha A. Wakim, Andre A. Wong, Tina Patel, Shyamal Coan, Kathryn Row, David Front Surg Surgery Desmoid type fibromatosis (DTF) is a rare benign tumor of connective tissue origin. While these tumors are typically not malignant, they can exhibit aggressive growth patterns causing mass effect on surrounding organs. These tumors typically present in the extremities and abdominal wall, rarely occurring in the mesentery, and abdominal organs. Due to the rarity of this tumor and the variable size and origin, it is difficult to provide exact prognosis, recurrence, and treatment efficacy regarding desmoid tumors arising from the ileocolic mesentery. We present a case of a young male with a sporadic 31 × 25 × 12 cm DTF arising from the ileocolic mesentery that was complicated by mass effect on bowel and intra-abdominal organs requiring surgical intervention. On presentation, the patient exhibited weight gain and abdominal pressure. Abdominal distension without tenderness on palpation was noted on physical examination. The tumor biopsy confirmed the diagnosis of DTF. No evidence of familial adenomatous polyposis or Gardner syndrome was identified. The tumor was surgically excised and intimately associated with the bowel requiring ileocolonic resection with primary anastomosis. At 3-months follow up, surveillance MRI showed no residual or recurrent lesion. A multi-disciplinary approach to this patient's diagnosis and treatment allowed for an accurate diagnosis, efficient treatment, and follow up plan. Frontiers Media S.A. 2020-09-02 /pmc/articles/PMC7493805/ /pubmed/33117845 http://dx.doi.org/10.3389/fsurg.2020.00060 Text en Copyright © 2020 Sioda, Wakim, Wong, Patel, Coan and Row. http://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
Sioda, Natasha A.
Wakim, Andre A.
Wong, Tina
Patel, Shyamal
Coan, Kathryn
Row, David
A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report
title A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report
title_full A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report
title_fullStr A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report
title_full_unstemmed A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report
title_short A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report
title_sort large sporadic intra-abdominal desmoid-type fibromatosis in a young male: a case report
topic Surgery
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7493805/
https://www.ncbi.nlm.nih.gov/pubmed/33117845
http://dx.doi.org/10.3389/fsurg.2020.00060
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