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Cancer prevention strategies for these less common cancers require accurate, age\u2010specific risk estimation. We pooled data from 4 LS research centers in a retrospective cohort study, to produce absolute incidence estimates for these cancer types, and to evaluate several potential risk modifiers. After elimination of 135 persons missing crucial information, cohort included 6,041 members of 261 families with LS\u2010associated <jats:italic>MLH1<\/jats:italic> or <jats:italic>MSH2<\/jats:italic> mutations. All were either mutation carriers by test, probable mutation carriers (endometrial\/colorectal cancer\u2010affected), or first\u2010degree relatives of these. Among mutation carriers and probable carriers, urologic tract cancer (<jats:italic>N<\/jats:italic> = 98) had an overall lifetime risk (to age 70) of 8.4% (95% CI: 6.6\u201310.8); risks were higher in males (<jats:italic>p<\/jats:italic> &lt; 0.02) and members of <jats:italic>MSH2<\/jats:italic> families (<jats:italic>p<\/jats:italic> &lt; 0.0001). Ovarian cancer (<jats:italic>N<\/jats:italic> = 72) had an lifetime risk of 6.7% (95% CI: 5.3\u20139.1); risks were higher in women born after the median year of birth (<jats:italic>p<\/jats:italic> &lt; 0.008) and in members of <jats:italic>MSH2<\/jats:italic> families (<jats:italic>p<\/jats:italic> &lt; 0.006). Brain tumors and cancers of the small bowel, stomach, breast and biliary tract were less common. Urologic tract cancer and ovarian cancer occur frequently enough in some LS subgroups to justify trials to evaluate promising prevention interventions. Other cancer types studied occur too infrequently to justify strenuous cancer control interventions. \u00a9 2008 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ijc.23508","type":"journal-article","created":{"date-parts":[[2008,4,8]],"date-time":"2008-04-08T16:46:40Z","timestamp":1207673200000},"page":"444-449","source":"Crossref","is-referenced-by-count":484,"title":["The risk of extra\u2010colonic, extra\u2010endometrial cancer in the Lynch syndrome"],"prefix":"10.1002","volume":"123","author":[{"given":"Patrice","family":"Watson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans F.A.","family":"Vasen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jukka\u2010Pekka","family":"Mecklin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inge","family":"Bernstein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markku","family":"Aarnio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heikki J.","family":"J\u00e4rvinen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Torben","family":"Myrh\u00f8j","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lone","family":"Sunde","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juul T.","family":"Wijnen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henry T.","family":"Lynch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2008,4,8]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19930201)71:3<677::AID-CNCR2820710305>3.0.CO;2-#"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0215(19990412)81:2<214::AID-IJC8>3.0.CO;2-L"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1086\/320610"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1023\/A:1006030116357"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2007.048991"},{"key":"e_1_2_7_7_2","volume-title":"The statistical analysis of failure time data","author":"Kalbfleisch JD","year":"1980"},{"key":"e_1_2_7_8_2","volume-title":"Survival analysis using the SAS system","author":"Allison P","year":"1995"},{"key":"e_1_2_7_9_2","unstructured":"Surveillance EaERSPwscgSSD. 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