In particular, several promising mutant IDH inhibitors including AG-120, IDH305, AG-881, BAY-1436032, AG-221, and FT-2120 have been developed and are currently in ongoing clinical trials for gliomas either alone or in combination with chemotherapy (NCT02073994, NCT02381886, NCT03343197, NCT02481154, NCT04164901, NCT02746081, NCT02273739, NCT03684811) [18]. Here, IDH2 is linked to central nervous system cancer.