Copyright
©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Sep 15, 2015; 7(9): 153-160
Published online Sep 15, 2015. doi: 10.4251/wjgo.v7.i9.153
Published online Sep 15, 2015. doi: 10.4251/wjgo.v7.i9.153
Hereditary diffuse gastric cancer: What the clinician should know
Ryan Ying Cong Tan, Joanne Ngeow, Cancer Genetics Service, Division of Medical Oncology, National Cancer Centre, Singapore 169610, Singapore
Author contributions: Tan RYC and Ngeow J equally contributed to this paper.
Supported by National Medical Research Council Transition Award (to Joanne Ngeow).
Conflict-of-interest statement: None.
Open-Access: 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. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Joanne Ngeow, MBBS, MRCP, MPH, Cancer Genetics Service, Division of Medical Oncology, National Cancer Centre, 11 Hospital Drive, Singapore 169610, Singapore. joanne.ngeow.y.y@singhealth.com.sg
Telephone: +65-64368172
Received: April 11, 2015
Peer-review started: April 12, 2015
First decision: May 18, 2015
Revised: June 2, 2015
Accepted: July 8, 2015
Article in press: July 10, 2015
Published online: September 15, 2015
Peer-review started: April 12, 2015
First decision: May 18, 2015
Revised: June 2, 2015
Accepted: July 8, 2015
Article in press: July 10, 2015
Published online: September 15, 2015
Core Tip
Core tip: While the incidence of hereditary diffuse gastric cancer remains low, it is an important clinical entity to recognize due to its high pathogenicity and penetrance. The International Gastric Cancer Linkage Consortium has outlined CDH1 testing criteria and developed clinical utility gene cards to help clinicians manage such patients. Significant progress has been made in recent years and in future, testing of other genes is likely for CDH1-negative families. The mainstay of treatment for asymptomatic carriers of CDH1 pathogenic mutations remains prophylactic total gastrectomy. Future research should focus on better risk stratification and surveillance methods.