Table 16-3ICG-HNPCC Definition of HNPCC (Lynch Syndrome)

Familial clustering of colorectal and/or endometrial cancer
Associated extracolonic cancers: cancer of the stomach, ovary, ureter/renal pelvis, brain, small bowel, hepatobiliary tract, and skin (sebaceous tumors)
Development of cancer at an early age
Development of multiple cancers
Features of colorectal cancer: (1) predilection for proximal colon; (2) improved survival; (3) multiple primary (synchronous/metachronous) colorectal cancers; (4) increased proportion of mucinous tumors, poorly differentiated tumors, and tumors with marked host-lymphocytic infiltration and lymphoid aggregation at the tumor margin
Features of colorectal adenoma: (1) the numbers vary from one to a few; (2) increased proportion of adenomas with a villous growth pattern and (3) probably rapid progression from adenoma to carcinoma
High frequency of MSI (MSI-H)
Immunohistochemistry: loss of hMLH1, hMSH2, or hMSH6 protein expression
Germ line mutation in MMR genes (hMSH2, hMLH1, hMSH3, hMSH6, hPMS1,hPMS2)

Reproduced with permission from Vasen et al. New clinical criteria for nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the International Collaborative Group on HNPCC. Gastroenterology 1999;116:1453-6.

From: Genetic Basis of Cancer Syndromes

Cover of Holland-Frei Cancer Medicine
Holland-Frei Cancer Medicine. 6th edition.
Kufe DW, Pollock RE, Weichselbaum RR, et al., editors.
Hamilton (ON): BC Decker; 2003.
Copyright © 2003, BC Decker Inc.

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