[Hereditary diffuse gastric cancer].

Hereditary diffuse gastric cancer is an autosomal dominant syndrome associated to CDH1 gene mutation that affects at least 30% of CDH1+ families, with a penetrance of 80% at 80 years, and multifocal signed ring cell in most of the cases. The suggested treatment is a prophylactic total gastrectomy with Roux-en-Y esophagojejunostomy, malignancy sing must be imperative a gastrectomy.