PMS2 and central nervous system cancer: Therefore, five glioma patients identified here with heterozygous GVs in genes conferring a high TMB, i.e., PMS2, POLE, and MUTYH, may be candidates for treatment with ICIs, such as nivolumab or pembrolizumab, shown to be effective in individual glioblastoma patients carrying homozygous PMS2 GVs or a heterozygous POLE GV [8, 31], and in a pediatric trial of refractory malignancies with high TMB including glioblastoma patients with biallelic PMS2 GVs [20].