Other Hereditary Cancers

There are many other cancer syndromes caused by inherited mutations. In general they are not increased in Jewish people compared to the general population.

  • Hereditary Diffuse Gastric Cancer/ Lobular Breast Cancer: Caused by mutation in the CDH1 gene associated with stomach, breast and colon cancer
  • Li Fraumeni Syndrome: hereditary mutation of the p53 gene associated with sarcomas, brain tumors, leukemia, adrenal and breast cancers.
  • Cowden’s Syndrome: hereditary mutation of the PTEN gene associated with breast cancer, thyroid (follicular) cancer and endometrial cancer
  • Von Hippel Lindau Syndrome: hereditary mutation of VHL gene associated with an increased risk of kidney cancer and benign blood vessel tumors
  • Multiple Endocrine Neoplasm Syndrome: associated with increased risk of tumors of the endocrine organs (pancreas, thyroid, pituitary, adrenal) which can be benign or malignant.

However, Ashkenazi Jews have higher rates of colorectal cancer (CRC) than any other ethnic group. In the general population, the lifetime risk of CRC is approximately five percent. In Ashkenazi Jews, this risk is two to three times greater. Approximately 10 percent of colorectal cancer is hereditary. Two known genetic syndromes account for a significant proportion of hereditary colorectal cancer: familial adenomatous polyposis (FAP) and Lynch syndrome, also called hereditary non-polyposis colon cancer (HNPCC). The mutations associated with these syndromes are inherited in an autosomal dominant pattern, which means affected individuals have a 50% chance of passing on the gene mutation to the next generation.

Familial Adenomatous Polyposis (FAP)
FAP accounts for approximately one percent of all colorectal cancer. Classic FAP is characterized by a preponderance of polyps, early age of onset and virtual certainty of developing colon cancer. It is caused by mutations in a tumor suppressor gene called APC. One specific APC mutation (I1307K) is found in approximately six percent of Ashkenazi Jews. This mutation does not cause classic FAP, but it does lead to a twofold increase in colon cancer risk. Ashkenazi Jews with I1307K APC mutations carry a 10 to 20% lifetime risk of colorectal cancer in carriers with no family history of CRC. Colon polyps may develop but in far fewer numbers than in classic FAP. The slight increase in risk of colon cancer in carriers does not warrant routine screening for this mutation or prophylactic surgery. 

Lynch Syndrome
Lynch syndrome accounts for approximately five percent of colorectal cancer cases. Lynch syndrome is sometimes referred to as hereditary non-polyposis colorectal cancer or HNPCC. It is caused by mutations in one of several genes that repair mismatches in DNA. People with this condition have a greatly increased chance of developing colorectal cancer and other cancers, especially at a young age. 

Signs you may be at risk for hereditary cancer:

  • Family history of early onset of cancer (diagnosis prior to age 50)
  • Several family members having the same type of cancer
  • Cancer occurring as a new tumor bilaterally in the same organ
  • Multiple types of cancer in the same person
  • Rare or unusual types of cancer
  • Related cancers occurring within multiple generations

What can I do to assess my hereditary cancer risk if I have a family history of cancer or a personal history of multiple cancers or cancer occurring at a young age?

  • Meet with a genetic counselor to evaluate your family history of cancer
  • Discuss the risks and benefits of genetic testing
  • Decide whether genetic testing is right for you

What are the benefits of knowing you have a hereditary cancer predisposition gene?
Genetic testing may not be right for everyone, but it can be helpful for you and your doctor to know about the increased health risks so that together you can determine a management plan that is right for you. This may include more frequent examinations, blood tests, and/or imaging, or risk-reducing surgeries. Some people choose not to undergo genetic tests but proceed with increased cancer surveillance as if they are at high risk.

Organizations That Specialize In Hereditary Cancers:

FORCE- Facing Our Risk of Cancer Empowered

Bright Pink

Sharsheret

Colon Cancer Alliance

Fight Colorectal Cancer

Still curious about hereditary cancer?
Feel free to contact the Center, our on-staff genetic counselor can work with you to answer your questions or to refer you to the appropriate organization or a genetic counselor in your area.

CJG-Whats-In-Your-Genes

Do You Know What's In Your Genes?

What is the most valuable gift you can give to your family? The gift of good health! There are many health conditions that run in families. Knowing your family health history can alert you to the potential risk for a variety of genetic disorders . Be sure to check with your relatives for warning signs and assess your risk for hereditary cancers!

Did you know: Ashkenazi Jews are 10 TIMES more likely to have BRCA mutations, which significantly increases lifetime risks for hereditary cancers, so what does this heightened risk mean for you? Click here to learn more !