Hereditary diffuse gastric cancer

Hereditary diffuse gastric cancer is an inherited genetic syndrome caused by a change in the E-cadherin gene.[1] It often results in stomach cancer such that it is recommended that those who carry the gene have their stomach removed.[1]

Genetics

Hereditary diffuse gastric cancer is inherited as an autosomal dominant mutation of the E-cadherin gene (CDH1), which is located on chromosome 16q22.1.

Treatment

Surgical removal of the stomach (gastrectomy) is typically recommended after for people after 20 years of age, and before 40 years of age.

Epidemiology

The median age at diagnosis is 38 years. Women are at higher risk for developing breast cancer.

References

  1. 1 2 World Cancer Report 2014. World Health Organization. 2014. pp. Chapter 5.4. ISBN 9283204298.
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