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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.
References:
-
Chung DC, Rustgi AK.
The hereditary
nonpolyposis
colorectal cancer
syndrome: genetics and
clinical implications.
Ann Intern Med. 2003
Apr 1;138(7):560-70.
[Medline]
-
Hampel H, Frankel WL,
Martin E, Arnold M,
Khanduja K, Kuebler P,
Nakagawa H, Sotamaa K,
Prior TW, Westman J,
Panescu J, Fix D,
Lockman J, Comeras I,
de la Chapelle A.
Screening for the
Lynch syndrome
(hereditary
nonpolyposis
colorectal cancer). N
Engl J Med. 2005 May
5;352(18):1851-60.
[Medline]
-
Pinol V, Castells A,
Andreu M,
Castellvi-Bel S,
Alenda C, Llor X,
Xicola RM, Rodriguez-Moranta
F, Paya A, Jover R,
Bessa X;
Gastrointestinal
Oncology Group of the
Spanish
Gastroenterological
Association. Accuracy
of revised Bethesda
guidelines,
microsatellite
instability, and
immunohistochemistry
for the identification
of patients with
hereditary
nonpolyposis
colorectal cancer.
JAMA. 2005 Apr
27;293(16):1986-94.
[Medline]
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