The 2021 WorldHealth Organization (WHO) classification (4)recognized classes of gliomas as “astrocytoma, IDH-mutant,”“oligodendroglioma, IDH-mutant, and 1p/19q-codeleted,” or“glioblastoma, IDH-wildtype.” Isocitrate dehydrogenase(IDH) mutations lead to the accumulation of the oncometabolite2-hydroxyglutarate, which is both a subject of ongoing research and a potentialtarget for emerging clinical interventions. This evidence concerns the gene IDH3A and central nervous system cancer.