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Hereditary diffuse gastric cancer is a rare, autosomal dominant hereditary cancer syndrome associated with germline mutations in CDH1 in which 60% -80% of affected individuals develop advanced diffuse gastric cancer, many as young adults. At clinical presentation, ~90% of these malignancies represent advanced, surgically incurable disease. As such, pre-symptomatic identification of germline CDH1 mutation carriers followed by early prophylactic total gastrictomy is the sole effective managementdoi:10.4236/jct.2013.42070 fatcat:6kjkgbiervdypiwulin75flfwe