GC has been reported to be one of the most frequent extracolonic malignancies in MSH2 and MLH1 mutation carriers with an estimated lifetime risk up to 13% and a frequency ranging from 1.6% in a Dutch registry to 3.1% in Korea and 10.9% in Finland, with a mean age of 56 years in Western countries and 46 years in Eastern countries [1,2,6]. The gene discussed is MSH2; the disease is gastric cancer.