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. 2019 Aug 31;11(1):119–131. doi: 10.1007/s13167-019-00186-x

Table 3.

Criteria for the diagnosis of hereditary non-polyposis CRC (HNPCC)

Guidelines Criteria Reference
Bethesda Guideline (1997) 1) Amsterdam criteria individuals [74]
2) Individuals with two HNPCC-related cancers: synchronous/metachronous colorectal cancers; endometrial, ovarian, gastric, hepatobiliary, small intestine, or renal tract transitional cell cancers

3) Individuals with colorectal cancer and a first-degree relative with one or more of the following:

(a) Colorectal cancer diagnosed under 45 years

(b) HNPCC-related cancer diagnosed under 45 years

(c) Adenoma diagnosed under 40 years

4) Individuals under 45 years of age with colorectal or endometrial cancer
5) Individuals with right-sided cancer of undifferentiated type
6) Individuals under 45 years of age with signet ring cancer
7) Individuals under 40 years of age with adenomas
Amsterdam Criteria-II (1999) 1) Three familial cases with HNPCC-associated cancer (CRC, cancer of small bowel, endometrium, ureter, or renal pelvis) in which two of the affected individuals are first-degree relatives of the third [75]
2) Colorectal cancers occurring in two successive generations
3) At least one colorectal cancer diagnosed under age 50 years
4) Familial adenomatous polyposis should be excluded in CRC cases
5) Tumors should be verified by a pathologist