- Hereditary Breast and Ovarian Cancer syndrome (HBOC)
- Lynch syndrome/Hereditary Non-Polyposis Colorectal Cancer (HNPCC)
- Familial Adenomatous Polyposis (FAP)/Attenuated Familial Adenomatous Polyposis (AFAP)
- MUTYH-associated Polyposis syndrome (MAP)
- MUTYH-associated Colon Cancer Risk
- Melanoma Cancer Syndrome (MCS)
- Li-Fraumeni Syndrome (LFS)
- PTEN Hamartoma Tumor syndrome (PHTS)
- Peutz-Jeghers Syndrome
- Hereditary Diffuse Gastric Cancer (HDGC) Syndrome
- Juvenile Polyposis Syndrome (JPS)
- Juvenile Polyposis Syndrome (JPS) and Hereditary Hemorrhagic Telangiectasia (HHT)
- PALB2-associated Cancer Risk
- CHEK2-associated Cancer Risk
- ATM-associated Cancer Risk
- NBN-associated Cancer Risk
- BARD1-associated Cancer Risk
- BRIP1-associated Cancer Risk
- RAD51C-associated Cancer Risk
- RAD51D-associated Cancer Risk
- Polymerase Proofreading-associated Syndrome (PPAS)
- Hereditary Mixed Polyposis Syndrome (HMPS)
Hereditary Diffuse Gastric Cancer Syndrome (HDGC) CDH1 ASSOCIATED CANCER RISKS
Hereditary Diffuse Gastric Cancer Syndrome (HDGC)
CDH1 ASSOCIATED CANCER RISKS
What does it mean to have a CDH1 gene mutation and a diagnosis of Hereditary Diffuse Gastric Cancer syndrome (HDGC)?
People with mutations in the CDH1 gene have a condition called Hereditary Diffuse Gastric Cancer syndrome, or HDGC. Men and women with HDGC have a high risk for stomach cancer. Women also have a high risk for breast cancer. These cancers can occur at young ages.
People with HDGC often get a kind of stomach cancer called “diffuse gastric cancer.” This type of stomach cancer is hard to find at early stages, because it does not form a noticeable tumor that can be easily seen with screening. For this reason, it is sometimes recommended that people with HDGC have their stomach removed before cancer can form. This may be the best choice for someone who has a CDH1 gene mutation and has one or more close relatives who have had stomach cancer in the past.
Women with HDGC have a high risk for a type of breast cancer that forms in the lobules of the breast. This is different than the more common type of breast cancer that starts in the milk ducts. It is recommended that women with CDH1 mutations start their breast screening at age 35 or earlier if they have relatives who had breast cancer at younger ages. It is also recommended that this screening include MRIs in addition to mammograms.
What can be done to protect people with HDGC from cancer?
Because people with HDGC have a high risk for the diffuse type of stomach cancer, screening may start in the teenage years. Stomach cancer screening is usually done by endoscopy, which involves putting a tube down the throat with a light and a camera so that doctors can look at the inside of the stomach for signs of cancer. However, the diffuse type of stomach cancer that occurs in people with HDGC is hard to see in this way, so it is recommended that the screening also include taking small tissue samples (biopsies) from the stomach to be examined under the microscope for signs of cancer.
Studies have shown that many stomach cancers are still missed, even with screening. For this reason, it is usually recommended that the stomach be removed completely as people with HDGC get older. Removing the stomach is a serious operation, but studies have shown that people can live long and healthy lives after the procedure.
Finally, there is concern that people with HDGC might have a higher than average risk for colon cancer. For this reason, it is recommended that people with HDGC start having colonoscopies at young ages.
We are still learning about CDH1 mutations and HDGC. Anyone who knows that they have a CDH1 gene mutation can benefit from being under the care of doctors and other health professionals who are knowledgeable about HDGC so that they can take steps to prevent cancer or find it as early as possible as well as have access to the most up-to-date information on HDGC and associated cancer risks.
Additional details about CDH1 gene mutations and HDGC, including information about the risks for different kinds of cancer, specific recommendations for medical care, and useful information for relatives of people who have a diagnosis of HDGC, are available within our Support Organizations page
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