Sometimes known as hereditary nonpolyposis colorectal cancer (HNPCC), Lynch syndrome is an inherited condition that increases the risk of many types of cancer, especially colorectal cancer. Doctors estimate that around 3 out of every 100 colon cancers or endometrial cancers are caused by Lynch syndrome.
Unfortunately, Lynch syndrome often goes undiagnosed, even when patients develop cancer. Mayo Clinic gastroenterologist and medical oncologist, Dr. Frank Sinicrope, M.D., emphasizes the importance for patients to know their family history of cancer, and discusses the criteria developed to help identify Lynch syndrome, and available treatment for those who develop cancer.
There are some particular features of Lynch syndrome-related colon cancer that are important to recognize – these cancers occur at a younger age of 45 - 60 years, as compared to 69 years for the typical colon cancer; cancers occur mostly in the right side of the colon, and it’s not uncommon to have more than one cancer found within the colon at the same time.
Dr. Sinicrope adds, “The most exciting news is that these cancers respond very well to immunotherapy. While the immune cells cannot kill tumor cells, we now have drugs known as checkpoint inhibitors that disable these proteins and enable immune system to kill cancer cells."
One such drug is pembrolizumab (Keytruda) shown to produce major responses in about fifty per cent of patients with Lynch syndrome-related cancer. This treatment has been approved by the FDA for patients with metastatic disease.
Learn more about ongoing research to evaluate immunotherapy in patients with colon cancer that has spread to lymph nodes around the tumor (stage III disease), at Mayo Clinic’s Gastrointestinal Cancer Program.
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