Given the well-known favorable outcome of diffuse IDH-mutant gliomas compared to malignant diffuse IDH-wt gliomas, on the one hand, and the known temozolomide-resistant phenotype of MMR-deficient cells, on the other hand, we addressed the question whether the clinical outcome of PMMRDIA significantly differs from that of other IDH-mutant gliomas. This evidence concerns the gene IDH2 and central nervous system cancer.