Hereditary diffuse gastric cancer (HDGC) is an autosomal-dominant cancer susceptibility syndrome characterized by early onset diffuse gastric cancer and lobular breast cancer.1 The clinical presentation of this particular form of familial gastric cancer was described more than 40 years ago2 and the phenotype can be dramatic. One descendant of the original Maori kindred from New Zealand died of gastric cancer at the age of 14 years; more than 25 of his relatives have succumbed to cancer.3 In a Michigan family with HDGC, 1 family member died from gastric cancer at the age of 17 years, and numerous other family members died of metastatic disease before reaching age 50 years.4 Fortunately, genetic testing has already transformed the care of families with HDGC, and the tragic outcomes in these 2 representative families can now largely be avoided. Prophylactic gastrectomy has the potential to eliminate the risk of gastric cancer in gene carriers,5- 7 while surveillance and other strategies for breast cancer prevention offer protection to women at risk.8
Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more
Subscribe for full-text access to content from 1998 forward and a host of useful features
Activate your current subscription (AMA members and current subscribers)
Purchase Online Access to this article for 24 hours
Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature
Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 5
Customize your page view by dragging & repositioning the boxes below.
More Listings atJAMACareerCenter.com >
Users' Guides to the Medical Literature
Sequencing the human genomeidentifying the entire sequence of base pairs in the 25 ...
All results at
and access these and other features:
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.