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. 2022 Oct 12;25(1):4–25. doi: 10.1093/neuonc/noac207

Table 2.

Targeted Therapies in Clinical Trial in Patients with IDH-mutant Glioma

Mechanism of action Therapy Phase Design Tumor type Trial
Treatment naive
IDH inhibitor Vorasidenib III • Randomized, placebo-controlled
• Primary outcome: PFS
• Non-enhancing tumors IDH-mutant glioma
• Within 5 years of initial surgery
• No prior RT or chemotherapy
NCT04164901
IDH inhibitor DS-1001 II • Primary outcomes: ORR, incidence of TEAEs • Treatment-naïve grade 2 IDH-mutant glioma NCT04458272
Glutaminase inhibitor Telaglenastat + RT and TMZ I • Determine MTD and RP2D • Grade 2 or 3 IDH-mutant glioma
• No prior RT or TMZ
NCT03528642
CDK4/6 inhibitor Palbociclib II • Non-randomized
• Primary outcomes: PFS-6
• Grade 3 oligodendroglioma (newly diagnosed and recurrent) NCT02530320
Recurrent after treatment
Demethylating agent ASTX7272 (decitabine + cedazuridine) 0/I • Phase I dose escalation to determine MTD
• Phase 0 surgical expansion for PD effects
• Recurrent IDH-mutant glioma
• Non-enhancing tumors
NCT03922555
Demethylating agent 5-azacytidine II • Single arm
• Primary outcome: PFS-6
• Recurrent grade 2 or 3 IDH-mutant glioma after standard treatment NCT03666559
PARP inhibitor Niraparib 0 • Phase 0 to examine PK, PD and presence of chromosomal fusion • Recurrent grade 2-4 IDH-mutant astrocytoma (Arm B only) NCT05076513
PARP inhibitor Pamiparib + metronomic TMZ I/II • Alkylator resistant and not-alkylator resistant arms in Phase II
• Surgical arm includes grade 4 tumors
• Primary outcomes: MTD and radiologic response
• Recurrent grade 2-4 IDH-mutant glioma NCT03914742
PPARP inhibitor Olaparib + Durvalumab II • Non-randomized
• Primary outcomes: ORR
• Recurrent IDH-mutant glioma at first or second relapse (Cohort A) NCT03991832
CDK4/6 inhibitor Palbociclib II • Non-randomized
• Primary outcomes: PFS-6
• Grade 3 oligodendroglioma (newly diagnosed and recurrent) NCT02530320
CDK4/6 inhibitor Abemaciclib II • Non-randomized, single arm
• Primary outcomes: PFS-6
• Recurrent oligodendroglioma NCT03220646
DHODH inhibitor BAY2402234 0 • Phase 0 for PD effects • Grade 4 recurrent IDH-mutant glioma Pending
Immune checkpoint inhibitor + IDH inhibitor Nivolumab + Ivosidenib II • Non-randomized, single arm
• Primary outcomes: PFS-6, ORR
• Recurrent IDH-mutant glioma
• Enhancing disease
NCT04056910