Conditions & Treatments

Ovarian Cancer and Hereditary Nonpolyposis Colon Cancer (HNPCC)

The risk for ovarian cancer is increased with hereditary nonpolyposis colon cancer (HNPCC), an autosomal dominant cancer genetic syndrome.

Ovarian Cancer as Part of Lynch Syndrome

A woman is at increased risk for ovarian cancer if she has Lynch syndrome, also called hereditary nonpolyposis colon cancer (HNPCC). Having this syndrome increases a woman's risk of having ovarian and uterine cancer at a much younger age than usual.

A clinical diagnosis of Lynch syndrome is made when the following characteristics are present in a family:

  • Three or more relatives with HNPCC-related cancer (colorectal, endometrial, ovarian, small bowel, stomach, liver, brain, upper respiratory tract, skin, prostate, gallbladder ducts, ureter, or renal pelvis)

  • Cancer affecting at least 2 successive generations

  • At least 1 case of HNPCC-related cancer diagnosed under age 50

  • A diagnosis of familial adenomatous polyposis has been ruled out

Some families with mutations in HNPCC-related genes may be tested even though they may not have all of the above characteristics. Genetic testing for Lynch syndrome should only be done after you and your doctor feel sure that it is the best thing for you and your family. It should be done by an expert counselor who can help you understand the results and what they may mean to you and your family.

The majority of Lynch syndrome cases are caused by mutations in 1 of several mismatch-repair genes. These mismatch-repair genes help correct "spelling errors" in DNA that happen during the cell division process. When these genes are altered, or mutated, then the "spelling errors" in the DNA cannot be repaired.

These errors in the DNA can lead to uncontrolled cell growth, which causes cancer. In Lynch syndrome, the germline mutation may be inherited from either the mother or the father and is present in all cells of the body. Whether a person who is born with a germline mutation will develop cancer, when he or she will develop cancer, and where the cancer(s) will appear depends on if and where (which cell type) a second mutation occurs. For example, if the second mutation is in the colon, then colon cancer may develop. If it is in the ovary, then ovarian cancer may develop. The process of tumor development actually requires mutations in multiple genes that regulate cell growth. What causes these additional mutations is generally unknown. Possible causes include chemical, physical, or biological environmental exposures or chance errors in DNA replication.

Some people who have inherited a germline mismatch-repair gene mutation never develop cancer because they never receive that second mutation to knock out the function of the gene and start the process of tumor formation. This can make the cancer appear to skip generations in a family, even when the mutation is present. People with a mutation, regardless of whether they develop cancer, have a 50/50 chance of passing the mutation on to each of their children.

It is important to remember that the mismatch-repair genes responsible for Lynch syndrome are not located on the sex chromosomes. Therefore, mutations can be inherited from the mother's or the father's side of the family.

Treatment Programs


Massachusetts General Hospital understands that a variety of factors influence patients' health care decisions. That's just one reason why we're dedicated to ensuring patients understand their diagnosis and treatment options. Because a single option might not serve all patients, we offer a wide range of coordinated treatments and related services across the hospital. Patients should consult with their primary care doctor or other qualified health care provider for medical advice and diagnosis information.

Select a treatment program for more information:



Cancer Center

  • Ovarian Cancer
    Access to promising new treatments at all stages of ovarian cancer.
Imaging

  • Pediatric Imaging
    The Pediatric Imaging Program at Massachusetts General Hospital Imaging specializes in ensuring the safety and comfort of child patients while providing the latest technology and the expertise of specialized pediatric radiologists.
  • Cancer Imaging and Intervention
    The Cancer Imaging and Intervention Program at Massachusetts General Hospital Imaging combines leading-edge technology and the expertise of specialty-trained radiologists to provide comprehensive cancer detection and monitoring, plus image-guided treatments for specific types of cancer.
Benson Henry Institute for Mind Body Medicine

  • Cancer Program
    Designed to supplement the medical treatment of cancer patients by giving them tools to cope with the stress of their illness, make informed choices about treatment, and maintain hope.

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