Comparison of the same gene between different groups revealed that there was no significant difference in the age distribution of patients harboring MLH1 and MSH2 germline P/LP variants among the three groups, while the age of patients harboring MSH6 (p = 0.005; p = 0.016) and PMS2 (p = 0.0019; p < 0.001) germline P/LP variants in the CNS LS-related tumor group was significantly lower than that in the other two groups. Here, PMS2 is linked to neoplasm.