Prophylactic Total Gastrectomy for Hereditary Diffuse Gastric Cancer

Germline mutations in the CDH1 gene that produces E-cadherin have been implicated in the development of early-onset diffuse gastric cancer, termed hereditary diffuse gastric cancer. The mean age of gastric cancer diagnosis in affected individuals is 37 years. By age 80, CDH1 mutation carriers who fulfill the clinical criteria for hereditary diffuse gastric cancer have an estimated lifetime risk of gastric cancer development of 67% for men and 83% for women. Data suggest that endoscopic surveillance for mutation carriers is largely ineffective. Prophylactic total gastrectomy between the ages of 18 and 40 years is recommended in carriers of germline-truncating E-cadherin mutations.