Syndromic gastric polyposis and hereditary gastric cancers

Abstract Although the majority of gastric carcinomas are sporadic neoplasms, approximately 10% show familial aggregation, and a hereditary cause is determined in 1–3% cases. Of these, hereditary diffuse gastric cancer (HDGC) is the most recognized predisposition syndrome. Although rare, some of the less commonly reported syndromes (including polyposis syndrome), also confer a markedly increased risk for development of gastric cancer. Identification and characterization of these syndromes requires a multidisciplinary effort involving oncologists, surgeons, genetic counselors, and pathologists.