While heterozygous GVs in POLE and PMS2 cause polymerase proofreading-associated polyposis or hereditary non-polyposis colorectal cancer, respectively, predisposing to colorectal cancer [58, 59], they have also been linked to glioma risk [5, 15, 32, 34, 60, 80], in line with our data. Here, PMS2 is linked to hereditary nonpolyposis colon cancer.