Zylberberg HM, Sultan K, Rubin S. Hereditary diffuse gastric cancer: One family’s story. World J Clin Cases 2018; 6(1): 1-5 [PMID: 29376063 DOI: 10.12998/wjcc.v6.i1.1]
Corresponding Author of This Article
Keith Sultan, MD, Assistant Professor, Division of Gastroenterology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 300 Community Dr., Manhasset, NY 11030, United States. ksultan@northwell.edu
Research Domain of This Article
Medicine, Research & Experimental
Article-Type of This Article
Case Report
Open-Access Policy of This Article
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/
World J Clin Cases. Jan 16, 2018; 6(1): 1-5 Published online Jan 16, 2018. doi: 10.12998/wjcc.v6.i1.1
Hereditary diffuse gastric cancer: One family’s story
Haley M Zylberberg, Keith Sultan, Steven Rubin
Haley M Zylberberg, Steven Rubin, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY 11549, United States
Keith Sultan, Division of Gastroenterology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Manhasset, NY 11030, United States
Steven Rubin, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Merrick, NY 11566, United States
Author contributions: Zylberberg HM, Sultan K and Rubin S designed the report; Zylberberg HM and Rubin S collected the patient’s clinical data; Zylberberg HM, Sultan K and Rubin S analyzed the data, drafted and edited the manuscript; all authors approve the final manuscript submitted and they approve the authorship list.
Informed consent statement: Informed consent was obtained from the patient by the article guarantor.
Conflict-of-interest statement: All authors declare that they have no conflicts of interest and nothing to declare.
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: Keith Sultan, MD, Assistant Professor, Division of Gastroenterology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 300 Community Dr., Manhasset, NY 11030, United States. ksultan@northwell.edu
Telephone: +1-516-3873990
Received: November 9, 2017 Peer-review started: November 9, 2017 First decision: November 30, 2017 Revised: December 8, 2017 Accepted: December 13, 2017 Article in press: December 13, 2017 Published online: January 16, 2018
Abstract
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.
Core tip: Individuals who carry the CDH1 gene mutation are at very high risk of acquiring hereditary diffuse gastric cancer, a cancer with a high mortality if not detected early. The clinical findings we describe in this case may aid medical practitioners in the assessment and testing of patients with a family history of gastric cancer and raise awareness about the importance of genetic testing for this condition.
