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Hereditary diffuse gastric cancer is an inherited condition, usually caused by a fault in the CDH1 gene, that carries a high risk of a type of stomach cancer that spreads through the stomach wall and of lobular breast cancer. Removing the whole stomach before cancer develops is the recommended option, and expert endoscopy checks can be offered to people who want to delay surgery.
Autosomal dominant. Largely caused by inactivating germline variants in the tumour suppressor CDH1 (E-cadherin); pathogenic CTNNA1 variants occur in a minority of families. Characterised by a high prevalence of diffuse gastric cancer and lobular breast cancer.
The 2020 International Gastric Cancer Linkage Consortium guidelines (Blair, Lancet Oncology 2020) relaxed the genetic testing criteria, mainly through less restrictive age limits; recommend prophylactic total gastrectomy as the gastric cancer risk management for pathogenic CDH1 variant carriers; and state increasing confidence that endoscopic surveillance in expert centres can be offered to those who wish to postpone surgery or whose gastric cancer risk is not well defined. The guideline also addresses the long-term effects of total gastrectomy in young patients.
In plain words · CDH1 (Cadherin-1) is a gene whose normal job is to hold cell growth in check. The public catalogues list it as a drug target, an oncogene driver, a tumour suppressor and a biomarker, and clinical evidence ties its variants to diagnosis, prognosis or drug response. Tied to Gastric & gastro-oesophageal junction cancer, Breast cancer, Ovarian cancer and 5 more.
Showing the target this term concerns: CDH1.
Shares Gastrectomy, Invitae Common Hereditary Cancers Panel, Endoscopy (EGD, EUS, ERCP), Hereditary cancer syndromes and the tag hereditary.
Shares Invitae Common Hereditary Cancers Panel, Endoscopy (EGD, EUS, ERCP), Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Invitae Common Hereditary Cancers Panel, Endoscopy (EGD, EUS, ERCP), Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Invitae Common Hereditary Cancers Panel, Endoscopy (EGD, EUS, ERCP), Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares MyRisk Hereditary Cancer (Myriad Genetics), Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares MyRisk Hereditary Cancer (Myriad Genetics), Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
Shares Hereditary cancer syndromes, Germline (hereditary) testing and the tag hereditary.
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