Hereditary diffuse gastric cancer

Adv Cancer Res. 2001;83:55-65. doi: 10.1016/s0065-230x(01)83002-5.


Hereditary diffuse gastric cancer (HDGC) is a cancer predisposition syndrome caused by germline mutation of the gene for the cell-to-cell adhesion protein E-cadherin. The syndrome is dominated by predisposition to the histologically diffuse, poorly differentiated form of gastric cancer. It is not associated with intestinal-type gastric cancer, but families may have an elevated risk of lobular breast cancer. Here, we review the identified families, mutations, and proposed mechanisms by which E-cadherin loss promotes tumorigenesis.

Publication types

  • Review

MeSH terms

  • Alleles
  • Carbohydrate Dehydrogenases / genetics
  • Family Health
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Male
  • Mutation*
  • Pedigree
  • Stomach Neoplasms / diagnosis*
  • Stomach Neoplasms / genetics*
  • Stomach Neoplasms / therapy*


  • Carbohydrate Dehydrogenases
  • cellobiose-quinone oxidoreductase