People with hereditary nonpolyposis colon cancer (HNPCC) have an 80 percent chance of developing colon cancer by their mid-70s. The disorder also increases a person's risk for cancers of the uterus, ovaries, small intestine, liver and brain. Appropriate screening can help prevent the development or spread of colon and other cancers.
At Mayo Clinic, experts from different specialties work together to address the needs of each patient at risk for HNPCC. Although HNPCC is quite rare, it is commonly treated at Mayo Clinic, one of the largest colon cancer treatment centers in the world. Mayo Clinic in Rochester, Minn., ranked No. 1 among U.S. hospitals in the specialty of digestive disorders in the 2007 U.S. News Best Hospitals ranking.
In addition to evaluating polyps and family history, genetic testing is available to help diagnose HNPCC and identify other family members with the disorder. The testing has some limitations, however. To date, researchers have identified four mutations that may contribute to this condition. But they believe many more may exist. Read more about hereditary nonpolyposis colon cancer diagnosis.
Treatment of HNPCC consists of screening for and removal of polyps (often performed during a colonoscopy procedure), along with monitoring for and (as needed) treating other associated cancers. Read more about hereditary nonpolyposis colon cancer treatment options.
Hereditary nonpolyposis colon cancer (HPNCC) is a hereditary form of colon cancer. It's called nonpolyposis to differentiate it from inherited colon cancers involving polyposis, or the development of hundreds to thousands of polyps. Although people with HPNCC still develop polyps, they don't develop as many.
The disease is also sometimes referred to Lynch syndrome. People with Lynch I syndrome usually develop a few polyps that have a high likelihood of becoming malignant. People with Lynch II syndrome are at risk for developing other tumors in addition to colon cancer.
The average age of colon cancer diagnosis for people with HNPCC is 45, compared to 65 in the general population. Two-thirds of colon cancers in those with HNPCC occur in the right side of the colon, whereas in the general population colon cancer occurs more frequently on the left side of the colon.
HNPCC results from a series of genetic mutations (abnormalities) and is autosomal dominant, meaning that only one parent must carry the mutated gene for a child (male or female) to have a 50 percent chance of inheriting the disorder.
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