PATHOLOGY OF HEREDITARY GASTRIC CANCER AND CLINICAL UTILITY OF ENDOSCOPY IN SCREENING AND SURVEILLANCE FOR HEREDITARY CANCER SYNDROMES

Abstract

Patients with hereditary gastric cancer syndromes account for 1-3% of patients with gastric cancer. Among hereditary gastric cancer syndromes, there are various genotype-phenotype features such as hereditary diffuse gastric cancer (HDGC), familial intestinal gastric cancer, gastric cancers associated with gastrointestinal polyposis syndromes, gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS), gastric cancer in gastrointestinal polyposis syndromes and others. Although endoscopy may be a useful tool to understand gastrointestinal phenotypes, the sensitivity of surveillance endoscopy is uncertain and thought to be poor for the detection of early disease in Western countries. However, some groups in Japan recently reported patients with HDGC in which the presence of HDGC had been suspected by endoscopic findings of multiple tiny pale areas. This may lead to standardization of endoscopic screening/surveillance system in the future. We herein review hereditary cancer syndromes associated with gastric cancer and will introduce several topics that were discussed at the 2019 IGCLC Hereditary Diffuse Gastric Cancer Consensus Clinical Guidelines Meeting held in New Zealand.