LS Diagnostic & Tumor Testing Criteria

A. Original Amsterdam criteria: Vasen HF, Mecklin JP, Khan PM, Lynch HT. The International Collaborative Group on Hereditary Non-polyposis Colorectal Cancer (ICG-HNPCC). Dis Colon Rectum. 1991;34:424-425. [Abstract]
[EXPAND Click Here for a Summary]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 age 50;
  • familial adenomatous polyposis should be excluded;
  • tumors should be verified by pathological examination.[/EXPAND]

B. Revised Amsterdam criteria: Vasen HF, Watson P, Mecklin JP, Lynch HT. New clinical criteria for hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the International Collaborative Group on HNPCC. Gastroenterology. 1999;116:1453-1456. [Abstract]
[EXPAND Click Here for a Summary]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 age 50;
  • familial adenomatous polyposis should be excluded;
  • tumors should be verified by pathological examination.[/EXPAND]

C. Original Bethesda criteria: Rodriguez-Bigas MA, Boland CR, Hamilton SR, et al. National Cancer Institute Workshop on Hereditary Nonpolyposis Colorectal Cancer Syndrome: meeting highlights and Bethesda guidelines. J Natl Cancer Inst. 1997; 89:1758-1762. [Abstract]
[EXPAND Click Here for a Summary]Bethesda Guidelines
1) Individuals with cancer in families that meet the Amsterdam criteria
2) Individuals with two HNPCC-related cancers, including synchronous and metachronous colorectal cancers or associated extracolonic cancers*
3) 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 <45y, and the adenoma diagnosed at age <40 y
4) Individuals with colorectal cancer or endometrial cancer diagnosed at age <45 y
5) Individuals with right-sided colorectal cancer with an undifferentiated pattern (solid/cribriform) on histopathology diagnosed at age <45y
6) Individuals with signet-ring-cell-type colorectal cancer diagnosed at age <45
7) Individuals with adenomas diagnosed at age <40 y
*Endometrial, ovarian, gastric, hepatobiliary, or small bowel cancer or transitional cell carcinoma of the renal pelvis or ureter[/EXPAND]

D. Revised Bethesda: Umar A, Boland CR, Terdiman JP, et al. Revised Bethesda Guidelines for hereditary nonpolyposis colorectal cancer (Lynch syndrome) and microsatellite instability. J Natl Cancer Inst. 2004; 96: 261-268. [Abstract]
[EXPAND Click Here for a Summary]

Revised Bethesda Guidelines
1) Individual with CRC dx <50
2) Individual with synchronous or metachronous CRC, or other HNPCC-associated tumors regardless of age
3) Individual with CRC & MSI-H histology dx <60
4) Individual with CRC & >1 FDR with an HNPCC-associated tumor, with one cancer dx <50
5) Individual with CRC & >2 FDRs or SDRs with an HNPCC-associated tumor, regardless of age[/EXPAND]