Hereditary diffuse gastric cancer: One family’s story

Hereditary diffuse gastric cancer (HDGC) is an inherited form of gastric cancer that carries a poor prognosis. Most HDGCs are caused by an autosomal dominant genetic mutation in the CDH1 gene, which carries a 70%-80% lifetime risk of gastric cancer. Given its submucosal origin, endoscopic surveillan...

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Detalles Bibliográficos
Autores principales: Zylberberg, Haley M, Sultan, Keith, Rubin, Steven
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5767847/
https://www.ncbi.nlm.nih.gov/pubmed/29376063
http://dx.doi.org/10.12998/wjcc.v6.i1.1
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author Zylberberg, Haley M
Sultan, Keith
Rubin, Steven
author_facet Zylberberg, Haley M
Sultan, Keith
Rubin, Steven
author_sort Zylberberg, Haley M
collection PubMed
description Hereditary diffuse gastric cancer (HDGC) is an inherited form of gastric cancer that carries a poor prognosis. Most HDGCs are caused by an autosomal dominant genetic mutation in the CDH1 gene, which carries a 70%-80% lifetime risk of gastric cancer. Given its submucosal origin, endoscopic surveillance is an unreliable means of early detection, and prophylactic gastrectomy is recommended for CDH1 positive individuals older than age 20 years. We describe the case of a male with recurrent gastric cancer who was diagnosed with HDGC secondary to the CDH1 mutation, and we also describe the patient’s pedigree and outcomes of recommended genetic testing.
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spelling pubmed-57678472018-01-28 Hereditary diffuse gastric cancer: One family’s story Zylberberg, Haley M Sultan, Keith Rubin, Steven World J Clin Cases Case Report Hereditary diffuse gastric cancer (HDGC) is an inherited form of gastric cancer that carries a poor prognosis. Most HDGCs are caused by an autosomal dominant genetic mutation in the CDH1 gene, which carries a 70%-80% lifetime risk of gastric cancer. Given its submucosal origin, endoscopic surveillance is an unreliable means of early detection, and prophylactic gastrectomy is recommended for CDH1 positive individuals older than age 20 years. We describe the case of a male with recurrent gastric cancer who was diagnosed with HDGC secondary to the CDH1 mutation, and we also describe the patient’s pedigree and outcomes of recommended genetic testing. Baishideng Publishing Group Inc 2018-01-16 2018-01-16 /pmc/articles/PMC5767847/ /pubmed/29376063 http://dx.doi.org/10.12998/wjcc.v6.i1.1 Text en ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial.
spellingShingle Case Report
Zylberberg, Haley M
Sultan, Keith
Rubin, Steven
Hereditary diffuse gastric cancer: One family’s story
title Hereditary diffuse gastric cancer: One family’s story
title_full Hereditary diffuse gastric cancer: One family’s story
title_fullStr Hereditary diffuse gastric cancer: One family’s story
title_full_unstemmed Hereditary diffuse gastric cancer: One family’s story
title_short Hereditary diffuse gastric cancer: One family’s story
title_sort hereditary diffuse gastric cancer: one family’s story
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5767847/
https://www.ncbi.nlm.nih.gov/pubmed/29376063
http://dx.doi.org/10.12998/wjcc.v6.i1.1
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