Prophylactic total gastrectomy for hereditary diffuse gastric cancer. Review of the literature.

Hereditary diffuse gastric cancer (HDGC) is characterized as an autosomal dominant cancer susceptibility syndrome largely attributable to germline mutations and deletions in the gene encoding E-cadherin, CDH1. Mutation carriers have a more than 70% lifetime risk of developing DGC and an elevated probability of lobular breast cancer. The aim of this review was to evaluate the results of surgical treatment for HDGC with special reference to the extent of its histological spread and to analyze the recent literature in order to provide an update on the current concepts of prophylactic gastrectomy for disease prevention. Nevertheless, it is not clear that our current knowledge of molecular and genetic diagnostics calls for the addition of HDGC to the roster of malignant familial syndromes in which early counseling and preventive surgical intervention should become the standard of care. Endoscopic screening cannot be recommended because the stomach appears normal and biopsies often fail to demonstrate signet ring cell adenocarcinoma. Prophylactic gastrectomy has provided many members of affected families with relief from GC with minimal implications.