Familial Cancer Syndrome Linked to Colorectal Cancer in Younger People

February 01, 1999

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 cancers. In addition, family members of individuals who develop colorectal cancer at an early age should now be monitored more closely," Dr. Guillem added.

The study also found that those with HNPCC had 13 times the rate of developing additional colorectal tumors compared with those in the non-HNPCC group (27% vs. 2%), supporting the recommendation for more extensive surgery to reduce the risk of developing a subsequent colorectal cancer in these patients.

While current guidelines recommend screening for colorectal cancer beginning at age 50 for average risk or at age 40 for those with a family history of the disease, individuals with HNPCC are advised to seek genetic counseling and screening with a colonoscopy every one to three years beginning at age 20.

"It is becoming increasingly important for people to know their family history and pay special attention to cancers that were detected at an early age," said Dr. Guillem. "Those individuals and their first-degree family members need to be screened more frequently and earlier than the general population for colorectal and other cancers," he added.

Memorial Sloan-Kettering maintains a registry of patients and their families who have early age-of-onset colorectal cancer or a history consistent with HNPCC and Familial Adenomatous Polyposis, as part of an international collaborative effort to identify, follow, study, and properly care for these individuals.
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Memorial Sloan-Kettering Cancer Center is the world's oldest and largest institution devoted to patient care, research, and education in cancer. Throughout its long, distinguished history, the Center has played a leadership role in defining the standards of care for patients with cancer. In 1998, Memorial Sloan-Kettering was named the nation's best cancer center for the sixth consecutive year by U.S. News & World Report.



Memorial Sloan Kettering Cancer Center

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