Prophylactic Total Gastrectomy: How Many?

Individuals with CDH1 germline mutations are at high risk of developing diffuse gastric cancer and prophylactic total gastrectomy represents the only life-saving treatment. Literature has reported a total of 224 surgical procedure in high-risk individuals, associated with germline CDH1 pathogenic mutations. The majority were described in the USA, the Netherlands, and Canada. Gastric tumor was identified in almost 85.4% of cases after prophylactic surgery, and a high rate of “no cancer” at histopathology was identified in the USA. Considering the mutation type, most of alterations were nonmissense versus missense sub-types. In this chapter, we will describe the penetrance risks for gastric cancer in CDH1 carriers and their implication for prophylactic gastrectomy.