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Diseases

Genetic and Rare Diseases Information Center (GARD)

Diffuse gastric cancer


Other Names for this Disease
  • Signet cell adenocarcinoma
  • Signet ring cell carcinoma
  • Signet ring cell gastric carcinoma
  • Signet ring gastric carcinoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My brother was diagnosed with signet ring cell gastric carcinoma. I was told that this is a genetic condition. Is testing available for this cancer? Who should I talk to if I want to be tested? Can excessive drinking cause it?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is diffuse gastric cancer?

Diffuse gastric cancer is a type of cancer found most often in the glandular cells lining the stomach, but can also develop in the bowel, breast, pancreas, bladder, prostate or lung. The term "signet ring cell" is often used because the cells look like signet rings when viewed under a microscope. The signet cells are a type of epithelial cell. Epithelial tissue is skin tissue, covering and lining the body both inside and out.[1] 
Last updated: 12/16/2011

Can diffuse gastric cancer be inherited?

Diffuse gastric cancer can be inherited or can happen sporadically in a family. Sporadic means that the cancer occurred randomly for the first time in a individual and was not inherited from a parent. Hereditary diffuse gastric cancer (HDGC) is caused by mutations in the CDH1 gene. Individuals with a CDH1 mutation typically develop cancer before age 40.[2]
Last updated: 12/16/2011

What is hereditary diffuse gastric cancer?

Hereditary diffuse gastric cancer (HDGC) is an inherited condition that is characterized by an increased risk of developing a specific form of stomach cancer (called diffuse gastric cancer). Women affected by this condition also have an increased risk for lobular breast cancer. Cancers associated with HDGC generally occur at younger ages than those seen in people who do not have a hereditary predisposition to cancer. HDGC is caused by changes (mutations) in the CDH1 gene and is inherited in an autosomal dominant manner. Management typically involves high-risk cancer screening and/or prophylactic surgeries.[3]   


Last updated: 3/17/2015

How is hereditary diffuse gastric cancer inherited?

Hereditary diffuse gastric cancer is inherited in an autosomal dominant manner.[3] This means that to be affected, a person only needs a change (mutation) in one copy of the responsible gene in each cell. In some cases, an affected person inherits the mutation from an affected parent. Other cases may result from new (de novo) mutations in the gene. These cases occur in people with no history of the disorder in their family. A person with hereditary diffuse gastric cancer has a 50% chance with each pregnancy of passing along the altered gene to his or her child.
Last updated: 3/17/2015

Is genetic testing available for hereditary diffuse gastric cancer?

Yes, genetic testing is available for CDH1, the gene known to cause hereditary diffuse gastric cancer.[3] Carrier testing for at-risk relatives and prenatal testing are possible if the disease-causing mutation in the family is known.

The Genetic Testing Registry (GTR) is a centralized online resource for information about genetic tests. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

Last updated: 3/17/2015

Who should I speak to if I am concerned about my inherited risks for diffuse gastric cancer?

We recommend that you contact a genetics clinic to discuss your concern with a genetic professional. Genetics clinics are a source of information for individuals and families regarding genetic conditions, treatment, inheritance, and genetic risks to other family members. More information about genetic consultations is available from Genetics Home Reference at http://ghr.nlm.nih.gov/handbook/consult. To find a genetics clinic, we recommend that you contact your primary healthcare provider for a referral.

The following online resources can help you find a genetics professional in your community:  
Last updated: 9/30/2011

Can diffuse gastric cancer be caused by excessive drinking?

Most of the time the exact cause of gastric cancer can not be determined; however there are many different factors that may put someone at an increased risk for developing stomach cancer. While it isn't clear if alcohol alone can increase this risk, it is thought that regular drinking may increase the risk in smokers.[4]

You can visit the following information pages develped by the National Cancer Insitute and Cancer Research UK to learn more about these risks.
http://www.cancer.gov/cancertopics/wyntk/stomach/page4
http://www.cancerhelp.org.uk/help/default.asp?page=3903
Last updated: 12/16/2011

References
Other Names for this Disease
  • Signet cell adenocarcinoma
  • Signet ring cell carcinoma
  • Signet ring cell gastric carcinoma
  • Signet ring gastric carcinoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.