Table 4

Hereditary non-polyposis colorectal cancer guidelines

*Endometrial, ovarian, gastric, hepatobiliary, or small bowel cancer or transitional cell carcinoma of the renal pelvis or ureter.
CRC, colorectal cancer; FDR, first degree relative; HNPCC, hereditary non-polyposis colon cancer syndrome; y, years.
Original Amsterdam criteria (Amsterdam criteria I)
There should be at least three relatives with colon cancer and
  • one should be a first degree relative of the other two;

  • at least two successive generations should be affected;

  • at least one should be diagnosed before the age of 50;

  • familial adenomatous polyposis should be excluded;

  • tumours should be verified by pathological examination.

Revised Amsterdam criteria (Amsterdam criteria II)
There should be at least three relatives with an HNPCC associated cancer (cancer of the colorectum, endometrium, small bowel, ureter, or renal pelvis) and
  • one should be a first degree relative to the other two;

  • at least two successive generations should be affected;

  • at least one should be diagnosed before the age of 50;

  • familial adenomatous polyposis should be excluded;

  • tumours should be verified by pathological examination.

Bethesda guidelines
  • Individuals with cancer in families that meet the Amsterdam criteria.

  • Individuals with two HNPCC related cancers, including synchronous and metachronous colorectal cancers or associated extracolonic cancers*

  • Individuals with colorectal cancer and a first degree relative with colorectal cancer and/or HNPCC related extracolonic cancer and/or a colorectal adenoma: one of the cancers diagnosed at age <45 y, and the adenoma diagnosed at age <40 y

  • Individuals with colorectal cancer or endometrial cancer diagnosed at age <45 y

  • Individuals with right sided colorectal cancer with an undifferentiated pattern (solid/cribriform) on histopathology diagnosed at age <45 y

  • Individuals with signet ring cell type colorectal cancer diagnosed at age <45

  • Individuals with adenomas diagnosed at age <40 y

AGA guidelines (Bethesda criteria–modified)
  • Amsterdam I criteria

  • Individuals with two HNPCC cancers (including synchronous/metachronous colorectal cancers)

  • Individuals with CRC and an FDR with CRC and/or HNPCC extracolonic cancer and/or colorectal adenoma (cancer <50 y and adenoma <40 y)

  • CRC or endometrial cancer <50 y

  • Right sided CRC with undifferentiated pattern on histology <50 y

  • Signet cell type CRC <50 y

  • Colorectal adenoma <40 y