Hereditary diffuse gastric cancer.
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.
Dunbier
A
A
Department of Biochemistry, University of Otago, Dunedin, New Zealand.
Guilford
P
P
D000483:Alleles; D002237:Carbohydrate Dehydrogenases / Q000235:genetics; D005192:Family Health; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D009154:Mutation; D010375:Pedigree; D013274:Stomach Neoplasms / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy*
D002237:Carbohydrate Dehydrogenases; C019859:cellobiose-quinone oxidoreductase