Low number of patients with path_MSH6 and path_PMS2 variants may reflect that they are infrequently identified by the Amsterdam or Bethesda criteria and are infrequently subjected to genetic testing.14 With the advent of universal screening of colorectal and endometrial cancers for LS, this situation is likely to change.6 We restricted our analysis to report the prospectively observed endometrial and ovarian cancer incidence and survival in women who had not had prophylactic RRS to provide a robust analysis of cancer risk and associated deaths using observational data from the PLSD. Here, MSH6 is linked to ovarian cancer.