Pathology of hereditary gastric cancer

Abstract

Hereditary gastric cancer includes the hereditary diffuse gastric cancer syndrome and a recently described syndrome designated as gastric adenocarcinoma and proximal polyposis of the stomach. Hereditary diffuse gastric cancer is caused by E-cadherin gene ( CDH1 ) alterations and a model of development has been described, encompassing mild non-atrophic gastritis, in situ signet ring cell (diffuse) carcinoma, pagetoid spread of signet ring cells and invasive carcinoma. Gastric adenocarcinoma and proximal polyposis of the stomach is characterized by fundic gland polyposis, with areas of dysplasia or intestinal type gastric cancer, restricted to the proximal stomach, with no evidence of colorectal or duodenal polyposis or other heritable gastrointestinal cancer syndromes. The genetic cause of gastric adenocarcinoma and proximal polyposis of the stomach has not been identifi ed yet.