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Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?

OBJECTIVE: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk pa...

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Autores principales: Campos, Fábio Guilherme, Martinez, Carlos Augusto Real, Bustamante-Lopez, Leonardo Alfonso, Mendonça, Roberta Laís da Silva, Kanno, Danillo Toshio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9723922/
https://www.ncbi.nlm.nih.gov/pubmed/36476966
http://dx.doi.org/10.1016/j.clinsp.2022.100144
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author Campos, Fábio Guilherme
Martinez, Carlos Augusto Real
Bustamante-Lopez, Leonardo Alfonso
Mendonça, Roberta Laís da Silva
Kanno, Danillo Toshio
author_facet Campos, Fábio Guilherme
Martinez, Carlos Augusto Real
Bustamante-Lopez, Leonardo Alfonso
Mendonça, Roberta Laís da Silva
Kanno, Danillo Toshio
author_sort Campos, Fábio Guilherme
collection PubMed
description OBJECTIVE: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. AIM: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. METHODS: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. RESULTS: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. CONCLUSION: : Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process.
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spelling pubmed-97239222022-12-07 Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development? Campos, Fábio Guilherme Martinez, Carlos Augusto Real Bustamante-Lopez, Leonardo Alfonso Mendonça, Roberta Laís da Silva Kanno, Danillo Toshio Clinics (Sao Paulo) Original Articles OBJECTIVE: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. AIM: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. METHODS: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. RESULTS: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. CONCLUSION: : Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process. Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo 2022-12-05 /pmc/articles/PMC9723922/ /pubmed/36476966 http://dx.doi.org/10.1016/j.clinsp.2022.100144 Text en © 2022 Published by Elsevier España, S.L.U. on behalf of HCFMUSP. https://creativecommons.org/licenses/by/4.0/This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Original Articles
Campos, Fábio Guilherme
Martinez, Carlos Augusto Real
Bustamante-Lopez, Leonardo Alfonso
Mendonça, Roberta Laís da Silva
Kanno, Danillo Toshio
Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?
title Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?
title_full Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?
title_fullStr Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?
title_full_unstemmed Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?
title_short Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?
title_sort intra-abdominal desmoid tumors in familial adenomatous polyposis: how much do clinical and surgical variables interfere with their development?
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9723922/
https://www.ncbi.nlm.nih.gov/pubmed/36476966
http://dx.doi.org/10.1016/j.clinsp.2022.100144
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