Hereditary Diffuse Gastric Cancer: A 2022 Update

Gastric cancer is ranked fifth among the most commonly diagnosed cancers, and is the fourth leading cause of cancer-related deaths worldwide. The majority of gastric cancers are sporadic, while only a small percentage, less than 1%, are hereditary. Hereditary diffuse gastric cancer (HDGC) is a rare...

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Main Authors: Christo Kole, Nikolaos Charalampakis, Stratigoula Sakellariou, George Papaxoinis, Konstantinos G. Apostolou, Nikolaos Machairas, Ioannis S. Papanikolaou, Dimitrios Schizas
Format: Article
Language:English
Published: MDPI AG 2022-12-01
Series:Journal of Personalized Medicine
Subjects:
Online Access:https://www.mdpi.com/2075-4426/12/12/2032
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author Christo Kole
Nikolaos Charalampakis
Stratigoula Sakellariou
George Papaxoinis
Konstantinos G. Apostolou
Nikolaos Machairas
Ioannis S. Papanikolaou
Dimitrios Schizas
author_facet Christo Kole
Nikolaos Charalampakis
Stratigoula Sakellariou
George Papaxoinis
Konstantinos G. Apostolou
Nikolaos Machairas
Ioannis S. Papanikolaou
Dimitrios Schizas
author_sort Christo Kole
collection DOAJ
description Gastric cancer is ranked fifth among the most commonly diagnosed cancers, and is the fourth leading cause of cancer-related deaths worldwide. The majority of gastric cancers are sporadic, while only a small percentage, less than 1%, are hereditary. Hereditary diffuse gastric cancer (HDGC) is a rare malignancy, characterized by early-onset, highly-penetrant autosomal dominant inheritance mainly of the germline alterations in the E-cadherin gene (<i>CDH1</i>) and β-catenin (<i>CTNNA1</i>). In the present study, we provide an overview on the molecular basis of HDGC and outline the essential elements of genetic counseling and surveillance. We further provide a practical summary of current guidelines on clinical management and treatment of individuals at risk and patients with early disease.
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spelling doaj.art-8511df3ec5824780aceeb5f3b06272cf2023-11-24T16:02:27ZengMDPI AGJournal of Personalized Medicine2075-44262022-12-011212203210.3390/jpm12122032Hereditary Diffuse Gastric Cancer: A 2022 UpdateChristo Kole0Nikolaos Charalampakis1Stratigoula Sakellariou2George Papaxoinis3Konstantinos G. Apostolou4Nikolaos Machairas5Ioannis S. Papanikolaou6Dimitrios Schizas7First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, 115 27 Athens, GreeceDepartment of Medical Oncology, Metaxa Cancer Hospital, 185 37 Athens, GreeceFirst Department of Pathology, National and Kapodistrian University of Athens, 115 27 Athens, GreeceSecond Department of Medical Oncology, Agios Savvas Anticancer Hospital, 115 22 Athens, GreeceSchool of Medicine, European University Cyprus, 2404 Nicosia, CyprusSecond Department of Propaedeutic Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, 115 27 Athens, GreeceHepatogastroenterology Unit, Second Department of Internal Medicine and Research Institute, National and Kapodistrian University of Athens, Attikon University Hospital, 124 62 Athens, GreeceFirst Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, 115 27 Athens, GreeceGastric cancer is ranked fifth among the most commonly diagnosed cancers, and is the fourth leading cause of cancer-related deaths worldwide. The majority of gastric cancers are sporadic, while only a small percentage, less than 1%, are hereditary. Hereditary diffuse gastric cancer (HDGC) is a rare malignancy, characterized by early-onset, highly-penetrant autosomal dominant inheritance mainly of the germline alterations in the E-cadherin gene (<i>CDH1</i>) and β-catenin (<i>CTNNA1</i>). In the present study, we provide an overview on the molecular basis of HDGC and outline the essential elements of genetic counseling and surveillance. We further provide a practical summary of current guidelines on clinical management and treatment of individuals at risk and patients with early disease.https://www.mdpi.com/2075-4426/12/12/2032hereditary diffuse gastric cancerCDH1CTNNA1MAP3K6gastrectomyclinical guidelines
spellingShingle Christo Kole
Nikolaos Charalampakis
Stratigoula Sakellariou
George Papaxoinis
Konstantinos G. Apostolou
Nikolaos Machairas
Ioannis S. Papanikolaou
Dimitrios Schizas
Hereditary Diffuse Gastric Cancer: A 2022 Update
Journal of Personalized Medicine
hereditary diffuse gastric cancer
CDH1
CTNNA1
MAP3K6
gastrectomy
clinical guidelines
title Hereditary Diffuse Gastric Cancer: A 2022 Update
title_full Hereditary Diffuse Gastric Cancer: A 2022 Update
title_fullStr Hereditary Diffuse Gastric Cancer: A 2022 Update
title_full_unstemmed Hereditary Diffuse Gastric Cancer: A 2022 Update
title_short Hereditary Diffuse Gastric Cancer: A 2022 Update
title_sort hereditary diffuse gastric cancer a 2022 update
topic hereditary diffuse gastric cancer
CDH1
CTNNA1
MAP3K6
gastrectomy
clinical guidelines
url https://www.mdpi.com/2075-4426/12/12/2032
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AT georgepapaxoinis hereditarydiffusegastriccancera2022update
AT konstantinosgapostolou hereditarydiffusegastriccancera2022update
AT nikolaosmachairas hereditarydiffusegastriccancera2022update
AT ioannisspapanikolaou hereditarydiffusegastriccancera2022update
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