Revised Criteria for Hereditary Non-Polyposis Colorectal Cancer (Lynch Syndrome)

Amsterdam Criteria (1991)

Three or more relatives with colorectal cancer, plus all of the following:

• One affected patient should be a first-degree relative of the other two;

• Colorectal cancer should involve at least two generations;

• At least one case of colorectal cancer should have been diagnosed before the age of 50 years.

Amsterdam II Criteria (Revised International Collaborative Group on Hereditary Non-Polyposis Colorectal Cancer – HNPCC (Criteria 1998)

Three or more relatives with HNPCC-associated cancer (colorectal cancer or cancer of the endometrium, small bowel, ureter or renal pelvis) plus all of the following:

• One affected patient should be a first-degree relative of the other two;

• Two or more successive generations should be affected;

• Cancer in one or more affected relatives should be diagnosed before the age of 50 years;

• Familial adenomatous polyposis should be excluded in any cases of colorectal cancer;

• Tumours should be verified by pathological examination.

Modified Amsterdam Criteria

Just one of these criteria need to be met:

• Very small families, which can not be further expanded, can be considered to have HNPCC with only two colorectal cancers in first-degree relatives if at least two generations have the cancer and at least one case of colorectal cancer was diagnosed by the age of 55 years;

• In families with two first-degree relatives affected by colorectal cancer, the presence of a third relative with an unusual early-onset neoplasm or endometrial cancer is sufficient.

Revised Bethesda Criteria (2003)

Just one these criteria need to be met:

• Diagnosed with colorectal cancer before the age of 50 years;

• Synchronous or metachronous colorectal or other HNPCC-related tumours (which include stomach, bladder, ureter, renal pelvis, biliary tract, brain (glioblastoma), sebaceous gland adenomas, keratoacanthomas and carcinoma of the small bowel), regardless of age;

• Colorectal cancer with a high-microsatellite instability morphology that was diagnosed before the age of 60 years;

• Colorectal cancer with one or more first-degree relatives with colorectal cancer or other HNPCC-related tumours. One of the cancers must have been diagnosed before the age of 50 years (this includes adenoma, which must have been diagnosed before the age of 40 years);

• Colorectal cancer with two or more relatives with colorectal cancer or other HNPCC-related tumours, regardless of age.

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