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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MDPI AG
2022-12-01
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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. |
first_indexed | 2024-03-09T16:12:59Z |
format | Article |
id | doaj.art-8511df3ec5824780aceeb5f3b06272cf |
institution | Directory Open Access Journal |
issn | 2075-4426 |
language | English |
last_indexed | 2024-03-09T16:12:59Z |
publishDate | 2022-12-01 |
publisher | MDPI AG |
record_format | Article |
series | Journal of Personalized Medicine |
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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