Clinical—Alimentary TractOne to 2-Year Surveillance Intervals Reduce Risk of Colorectal Cancer in Families With Lynch Syndrome
Section snippets
Dutch Lynch Syndrome Registry
In 1987, a national registry for families with hereditary CRC was established in The Netherlands. The registry had three objectives: (1) to promote surveillance in families at risk for CRC; (2) to guarantee the continuity of the surveillance program; and (3) to promote research in the field. The methods and approach of the registry have been described elsewhere.10 In brief, clinical specialists or clinical genetics centers from all parts of The Netherlands refer to the registry as any family
Surveillance of Lynch Syndrome Families
A total of 745 MMR mutation carriers (male 308) were included. The characteristics of this study group are shown in Table 1. The carriers belong to 205 families with a pathogenic MMR mutation. Seventy-five of the families harbored an MLH1 mutation, 87 an MSH2 mutation, and 43 an MSH6 mutation.
The mean follow-up was 7.2 years (range, 0.4–13.7 years). The mean surveillance interval was 16 months. Thirty-three patients (4.4%) developed CRC under surveillance. The age at diagnosis of CRC in these
Discussion
The present study demonstrates that carriers of an MMR gene mutation have a relatively low risk of developing CRC with 2 years' surveillance intervals. Although the risk of CRC was substantially higher in carriers ≥40 years of age compared with carriers <40 years and the risk was higher in carriers of MLH1 and MSH2 mutations compared with carriers of MSH6 mutations, the differences were only of borderline significance. Relatives of non-LS families have a significantly lower risk of developing
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Conflicts of interest The authors disclose no conflicts.