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Familial gastric cancer

Orpha number ORPHA26106
Synonym(s) Familial stomach cancer
Prevalence Unknown
Inheritance Multigenic/multifactorial
Age of onset Adult
ICD-10
  • C16
ICD-O -
OMIM
UMLS -
MeSH -
MedDRA -

Summary

Familial gastric cancer is a heterogeneous disease suspected when two first degree relatives are affected with stomach cancer before 50 years of age or when at least three people in a family are affected, regardless of their relation and age. Cases of stomach cancer are in spectacular regression, mainly thanks to the improved conditions of food preservation. However, the risk of stomach cancer is increased in individuals with certain inherited diseases, mainly hereditary non-polyposis colorectal cancer syndrome (HNPCC, 19% of patients) and familial stomach cancer. An increased risk is also associated with digestive polyposis as the adenoma may degenerate and infiltrate gastric polyps. When it is associated with a constitutive mutation in the cadherin E (CDH1)gene (mutation of the CDH1 gene is found in about 25% of cases), the disease also increases the risk for cancer of the colon at a young age. People at risk in these families should undergo yearly gastroscopies and/or echoendoscopies after 25 years of age. However, because of the recent nature of the syndrome, there is no consensual recommendation for patient management. Prophylactic total gastrectomy is currently under careful consideration.

Expert reviewer(s)

  • Dr Sylviane OLSCHWANG

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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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