In patients with IDH-mutant gliomas, the efficacy of novel therapies targeting the mutant IDH enzyme using oral IDH1-inhibitors (e.g., ivosidenib), IDH2-inhibitors such as endasidenib, inhibitors of both IDH1 and IDH2 mutations (e.g., vorasidenib), and vaccines targeting the IDH1 (R132H) neoepitope are currently under investigation. Here, IDH2 is linked to central nervous system cancer.