Skip to
  1. Homepage
  2. Rare diseases
  3. Search
Simple search

Simple search

(*) mandatory field


Other search option(s)

Familial gastric cancer

Synonym(s) Familial stomach cancer
Prevalence Unknown
Inheritance Multigenic/multifactorial
Age of onset Adult
  • C16
MeSH -
MedDRA -


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

(*) Required fields.

Attention: Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

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)
Get Acrobat Reader
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.