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Any Lynch cancer 76 percent* 80 percent* 73 percent* Colorectal 58 to 65 percent 50 to 53 percent 54 to 63 percent 39 to 68 percent 36 to 69 percent 18 to 30 percent Endometrial NA 27 percent NA 40 percent NA 71 percent Ovarian NA 6 percent NA 12 percent ? Upper urologic tract 2.1 percent 0.4 percent 20 percent 9 percent ? Gastric 6 percent* 5 percent* ? Small bowel • 3 percent 6 percent 3 percent 6 percent ? Biliary/pancreatic 4 percent • ? Brain tumors (gliomas) 1.7 percent 2.5 percent ? Sebaceous gland tumors 42 percent of families Δ 44 percent of families Δ 0 percent of families Δ NA: not applicable; ?: unknown. * Not reported separately by sex. • Not reported separately by genotype. Δ Percent of Lynch families with one or more members with sebaceous gland tumors. Data from: Hampel H, Stephens J, Pukkala E, et al. Cancer risk in hereditary nonpolyposis colorectal cancer syndrome: later age of onset. Gastroenterology 2005; 129:415. Watson P, Vasen HFA, Mecklin JP, et al. The risk of extra-colonic, extra-endometrial cancer in the Lynch syndrome. Int J Cancer 2008; 123:444. Koessler T, Ostergaard MZ, Song H, et al. Common variants in mismatch repair genes and risk of colorectal cancer. Gut 2008; 57:1097. Hendriks YM, Wagner A, Morreau H, et al. Cancer risk in hereditary nonpolyposis colorectal cancer due to MSH6 mutations: impact on counseling and surveillance. Gastroenterology 2004; 127:17. South DC, Hampel H, Comeras I, et al. The frequency of Muir-Torre syndrome among Lynch syndrome families. J Natl Cancer Inst 2008; 100:277. Barrow E, Alduaij W, Robinson L, et al. Colorectal cancer in HNPCC: cumulative lifetime incidence, survival and tumour distribution. A report of 121 families with proven mutations. Clin Genet 2008; 74:233.