Onkologie. 2026:20(2):91-97 | DOI: 10.36290/xon.2026.017

Vorasidenib - a revolution in the treatment paradigm for low-grade gliomas

Martin Palkovský, Štěpánka Hauserová, Renata Soumarová
Onkologická klinika, 3. LF UK a FNKV, Praha

Gliomas are the most common primary brain tumors in adults and are characterized by high intratumoral and interindividual heterogeneity. The latest WHO classification from 2021 divides glial tumors of the central nervous system (CNS) into two basic groups according to the presence or absence of mutations in the isocitrate dehydrogenase 1 and 2 (IDH1/2) genes. Diffuse gliomas in adults with IDH mutations are further divided into astrocytomas grade 2, 3, or 4 and oligodendrogliomas with IDH mutations and 1p/19q codeletion grade 2 or 3. The product of mutated IDH genes, D-2-hydroxyglutarate (D-2-HG), induces DNA hypermethylation and thus stimulates tumor growth. IDH1/2 inhibitors, such as ivosidenib and vorasidenib, reduce D-2-HG levels and improve progression free survival in clinical trials. The phase III INDIGO study demonstrated the superiority of postoperatively administered vorasidenib, a dual IDH1/2 inhibitor that penetrates the brain, over placebo in patients with non-enhan­cing grade 2 gliomas. In this review, we describe the development of IDH inhibitors in gliomas and the impact of the INDIGO study on changing the current treatment strategy for low-grade gliomas.

Keywords: gliomas, CNS, ivosidenib, vorasidenib, IDH1, IDH2, INDIGO.

Accepted: April 20, 2026; Published: April 28, 2026  Show citation

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Palkovský M, Hauserová Š, Soumarová R. Vorasidenib - a revolution in the treatment paradigm for low-grade gliomas. Onkologie. 2026;20(2):91-97. doi: 10.36290/xon.2026.017.
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