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IDH1 R132H — defines IDH-mutant astrocytoma (WHO 2021 grade 2-4) and oligodendroglioma (w...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDBMA-IDH1-R132H-GBM
TypeActionability
Statusreviewed 2026-04-27 | pending_clinical_signoff | actionability review required
DiseasesDIS-GBM
SourcesSRC-CIVIC SRC-EANO-GBM-2024 SRC-NCCN-CNS-2025

Actionability Facts

BiomarkerBIO-IDH-MUTATION
VariantIDH1 R132H
DiseaseDIS-GBM
ESCAT tierIIA
Recommended combinationsvorasidenib monotherapy (low-grade IDH-mut), ivosidenib (high-grade IDH-mut, off-label/trial)
Evidence summaryIDH1 R132H — defines IDH-mutant astrocytoma (WHO 2021 grade 2-4) and oligodendroglioma (with 1p/19q codeletion). Vorasidenib improved PFS in residual/recurrent grade 2 IDH-mut glioma post- resection (INDIGO, Mellinghoff et al. NEJM 2023) — FDA-approved 2024. For grade 4 (IDH-mut GBM equivalent) — standard of care still RT + TMZ; ivosidenib explored.

Notes

ESCAT IIA. OncoKB Level 2. IDH-mut GBM is a distinct entity from IDH-WT GBM and has better prognosis. R132H IHC is the standard screening test; non-canonical R132 require sequencing.

Used By

No reverse references found in the YAML corpus.