Amsterdam criteria
Encyclopedia
The Amsterdam criteria are a set of diagnostic criteria used by doctors to help identify families which are likely to have Lynch syndrome, also known as hereditary nonpolyposis colorectal cancer (HNPCC).

The Amsterdam criteria arose as a result of a meeting of the International Collaborative Group on Hereditary Non-Polyposis Colon Cancer in Amsterdam, in 1990. Following this, some of the genetic mechanisms underlying Lynch syndrome were elucidated during the 1990s and the significance of tumours outside the colon
Colon (anatomy)
The colon is the last part of the digestive system in most vertebrates; it extracts water and salt from solid wastes before they are eliminated from the body, and is the site in which flora-aided fermentation of unabsorbed material occurs. Unlike the small intestine, the colon does not play a...

, such as those of the endometrium
Endometrium
-Function:The endometrium is the innermost glandular layer and functions as a lining for the uterus, preventing adhesions between the opposed walls of the myometrium, thereby maintaining the patency of the uterine cavity. During the menstrual cycle or estrous cycle, the endometrium grows to a...

, small intestine
Small intestine
The small intestine is the part of the gastrointestinal tract following the stomach and followed by the large intestine, and is where much of the digestion and absorption of food takes place. In invertebrates such as worms, the terms "gastrointestinal tract" and "large intestine" are often used to...

 and ureter
Ureter
In human anatomy, the ureters are muscular tubes that propel urine from the kidneys to the urinary bladder. In the adult, the ureters are usually long and ~3-4 mm in diameter....

, became more clear. These changes in the knowledge of the syndrome lead to a revision of the Amsterdam criteria and were published in Gastroenterology
Gastroenterology (journal)
Gastroenterology is the official medical journal of the American Gastroenterological Association. Its first issue was published in 1943. It is currently published by Elsevier. According to the Journal Citation Reports, the journal has a 2010 impact factor of 12.032, ranking it first out of 71...

 journal in 1999.

Criteria

The initial Amsterdam criteria were a series of clinical criteria that were colloquially known as the ‘‘3-2-1’’ rule. They were formulated to serve as a common starting point for future research into the genetics underlying the disease. The criteria were as follows:
  1. At least 3 relatives with histologically confirmed colorectal cancer, 1 of whom is a first degree relative of the other 2; familial adenomatous polyposis
    Familial adenomatous polyposis
    Familial adenomatous polyposis is an inherited condition in which numerous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colon cancer occurs when not treated....

     should be excluded;
  2. At least 2 successive generations involved;
  3. At least 1 of the cancers diagnosed before age 50.


These criteria were found to be too strict and were expanded to include the associated non-colorectal cancers in 1998. These were called the Amsterdam II clinical criteria for families with Lynch syndrome.

Each of the following criteria must be fulfilled:
  • 3 or more relatives with an associated cancer (colorectal cancer
    Colorectal cancer
    Colorectal cancer, commonly known as bowel cancer, is a cancer caused by uncontrolled cell growth , in the colon, rectum, or vermiform appendix. Colorectal cancer is clinically distinct from anal cancer, which affects the anus....

    , or cancer of the endometrium
    Endometrium
    -Function:The endometrium is the innermost glandular layer and functions as a lining for the uterus, preventing adhesions between the opposed walls of the myometrium, thereby maintaining the patency of the uterine cavity. During the menstrual cycle or estrous cycle, the endometrium grows to a...

    , small intestine, ureter or renal pelvis);
  • 2 or more successive generations affected;
  • 1 or more relatives diagnosed before the age of 50 years;
  • 1 should be a first-degree relative of the other two;
  • Familial adenomatous polyposis
    Familial adenomatous polyposis
    Familial adenomatous polyposis is an inherited condition in which numerous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colon cancer occurs when not treated....

     (FAP) should be excluded in cases of colorectal carcinoma;
  • Tumors should be verified by pathologic examination.

Alternatives

In 1997, the National Cancer Institute
National Cancer Institute
The National Cancer Institute is part of the National Institutes of Health , which is one of 11 agencies that are part of the U.S. Department of Health and Human Services. The NCI coordinates the U.S...

 published a set of recommendations called the Bethesda guidelines for the identification of individuals who should receive genetic testing for Lynch syndrome related tumors. The NCI revisited and revised these criteria in 2004.

The Revised Bethesda Guidelines are as follows:
  • Colorectal carcinoma (CRC) diagnosed in a patient who is less than 50 years old;
  • Presence of synchronous (at the same time) or metachronous (at another time i.e.- a re-occurrence of) CRC or other Lynch syndrome-associated tumors, regardless of age;
  • CRC with high microsatellite instability
    Microsatellite instability
    Microsatellites are repeated sequences of DNA. Although the length of these microsatellites is highly variable from person to person, each individual has microsatellites of a set length. These repeated sequences are common, and normal...

     histology
    Histology
    Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...

    diagnosed in a patient less than 60 years old;
  • CRC diagnosed in one or more first-degree relatives with a Lynch syndrome-associated tumor, with one of the cancers being diagnosed at less than 50 years of age;
  • CRC diagnosed in two or more first-degree or second-degree relatives with Lynch syndrome-associated tumors, regardless of age.


The Revised Bethesda Guidelines have been reported as being more accurate than the Amsterdam II Criteria in detecting individuals and families at risk of Lynch syndrome.
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