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Hereditary diffuse gastric cancer

ORPHA26106
Synonym(s) FDGC
Familial diffuse cancer of stomach
Familial diffuse gastric cancer
HDGC
Hereditary diffuse cancer of stomach
Hereditary diffuse gastric adenocarcinoma
Prevalence Unknown
Inheritance Autosomal dominant
Age of onset Adult
ICD-10
  • C16
OMIM
UMLS -
MeSH -
MedDRA -

Summary

Familial gastric cancer refers to the occurrence of gastric cancer in a familial context and is described as two or more cases of gastric cancer in first or second degree relatives with at least one case diagnosed before the age of 50. Familial clustering is observed in 10% of all cases of gastric cancer, and includes hereditary diffuse gastric cancer (early onset diffuse-type gastric cancer), familial intestinal gastric cancer (familial clustering of intestinal type gastric adenocarcinoma), and gastric adenocarcinoma and proximal polyposis of the stomach (see this term). Familial gastric cancer can also occur in other hereditary cancer syndromes such as Lynch syndrome, Li-Fraumeni syndrome, familial adenomatous polyposis and juvenile polyposis syndrome (see these terms).


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Detailed information

Review article
  • EN (2009)
  • IT (2009)
Clinical practice guidelines
  • FR (2011)
Guidance for genetic testing
  • EN (2013,pdf)
Article for general public
  • FR (2014)
  • IT (2009)
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