Summary: | <p>Abstract</p> <p>Hereditary diffuse gastric cancer (HDGC) is the only known predisposition syndrome dominated by carcinoma of the stomach and with a recognised genetic cause. Germline mutations in the E-cadherin gene (<it>CDH1</it>) co-segregate with the disease in about half of the families with multiple diffuse gastric cancer. In these families, identification of the <it>CDH1 </it>mutation allows for clinical measures to be taken. Importantly, clinical intervention is likely to be therapeutic and associated with tolerable morbidity. This review is thus aimed at providing a current overview of the clinical management and the underlying biology of HDGC.</p>
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