In the present study, nine families (1.9%) fulfilled the Amsterdam II criteria and of these seven (1.5%) had mutation-verified LS: three with mutations in MLH1 and four in MSH2. This correlates well with other studies on unselected uterine cancer cases, which have found LS in 1.8-4% [11,30-32], while LS was found in 9% of uterine cancer cases diagnosed before the age of 50 years [10]. The gene discussed is MSH2; the disease is uterine cancer.