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These risks are consistent with previously reported estimates, 2 , 12 , 15 except that the birth year cohort difference has not been previously reported to our knowledge. Substantially and significantly different risks were seen among registries, with Finland and Denmark having higher incidence rates than the other registries. Lack of primary data has hampered consensus development regarding prevention strategies for these less common LS tumors. Secondary analyses excluding bladder cancers from the UTC diagnoses had little effect on differences among subgroups although, of course, lifetime risk estimates were reduced. In addition, we evaluated the hypothesis that there are differences in incidence between MLH1 and MSH2 mutation carriers, and that, after adjustment for family size, there is significant interfamily variability in incidence rates. The cancer types designated in this study combine several significantly different cancer subtypes, such as bladder cancer and ureteral cancer. and small bowel adenocarcinoma and small bowel carcinoid. Lifetime risk estimates are provided in Table III. We concluded it was impractical and unnecessary albeit desirable to make estimates for all specific cancer subtypes. No special breast cancer surveillance is indicated for LS patients. Gastric cancer had the third highest incidence among the cancers we studied. After eliminating individuals who were missing crucial data gender, year of birth, year of death , 6, remained for analysis. Among the progenitors' descendants, we excluded all mutation noncarriers by test or inference i. This study was not designed to provide an accurate idea of the relative risk of these cancers.

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Description: Mutation carrier status based on mutation test results only. The decision to recommend a specific cancer prevention strategy, whether it is screening for early cancer detection, prophylactic surgery or chemoprevention, depends on many factors. In the case of small bowel cancer, it has been noted that new, less invasive technologies for evaluation of the small bowel are becoming available. UTC risk observed in this cohort was markedly higher than that observed in a previous study of the Finnish families 2 and comparable to that observed in a previous study of the Dutch families. Some of these cases were used in previous studies. Further studies are needed to identify the clinical features of UTC, so that an optimal prevention strategy can be developed and evaluated. Results of these analyses are given in Table II. Each center provided data on all eligible families and on all eligible members of those families. These risks are consistent with previously reported estimates, 2 , 12 , 15 except that the birth year cohort difference has not been previously reported to our knowledge. MSH2 , males versus females, before versus after midpoint year of birth and registry. Urologic tract cancer and ovarian cancer occur frequently enough in some LS subgroups to justify trials to evaluate promising prevention interventions. The initial cohort included 6, members of families. Epithelial upper urologic tract cancer is more common than bladder cancer in the LS, 1 and upper urologic tract cancer rates are similar in all 4 countries.
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