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Familial Cancer Syndrome Linked to Colorectal Cancer in Younger People

Important Factor in Limiting Risk of Additional Cancers in Patients and their Family Members

New York, January 28, 1999 -- Nearly one-fifth of patients who develop colorectal cancer at a young age (40 and younger) have a family history consistent with a familial colorectal cancer syndrome known as hereditary non-polyposis colorectal cancer (HNPCC), according to researchers at Memorial Sloan-Kettering Cancer Center.

The study, published in the current issue of Diseases of the Colon and Rectum, looked at 230 patients 40 years of age and younger who were diagnosed with colorectal cancer and found that nearly 20 percent of them had the HNPCC syndrome.

HNPCC is a dominantly inherited disorder, accounting for about five percent of all colorectal cancer cases. It is generally a fast-growing cancer that affects people primarily in their 40s. People who carry the genetic mutation for HNPCC have an 80 to 85 percent lifetime risk of developing colorectal cancer and are at increased risk of developing endometrial, ovarian, stomach, kidney, and other cancers. The criteria for a clinical diagnosis of HNPCC include: three first-degree relatives with colorectal cancer, spanning two generations, one of whom is less than 50 years of age at the time of diagnosis. This would exclude those with another type of inherited colorectal cancer called Familial Adenomatous Polyposis, a condition where people develop hundreds of colon polyps at a very young age.

"Individuals who develop colorectal cancer at a young age require special surveillance to protect them from developing other types of cancers," said Dr. Jose Guillem, Associate Attending Surgeon at Memorial Sloan-Kettering Cancer Center and lead author of the study. "By identifying and tracking individuals with HNPCC, we can intervene at an early stage and make appropriate, and where warranted, aggressive treatment decisions to prevent the development of additional colorectal and other cance
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Contact: Stacey Harris
harriss@mskcc.org
212-639-3573
Memorial Sloan-Kettering Cancer Center
1-Feb-1999


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