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Low-grade glioma (LGG, WHO grade 2 — IDH-mutant)

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IDDIS-GLIOMA-LOW-GRADE
TypeMaladie
Alias
Astrocytome diffus IDH mutantLGGGliome de bas gradeOligodendroglioma IDH-mutant 1p/19q-codéléГліома низького ступеня (WHO grade 2 — IDH-мутантна)
Étatreviewed 2026-04-27 | pending_clinical_signoff
MaladiesAucune déclaration
SourcesSRC-EANO-GBM-2024 SRC-NCCN-CNS-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Source YAML

icd_o_3_morphology9400/3
icd_o_3_topography["C71"]
icd_10C71
who_classificationWHO CNS 5th ed (2021): Astrocytoma IDH-mutant grade 2 (9400/3) + Oligodendroglioma IDH-mutant 1p/19q-codeleted grade 2 (9450/3)

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Notes

Defined molecularly, not just histologically: IDH-mutant grade 2 diffuse astrocytoma + IDH-mutant 1p/19q-codeleted oligodendroglioma. IDH-wild-type "diffuse astrocytomas" are reclassified as molecular GBM (DIS-GBM) per WHO 2021 even at grade 2 histology. Standard care: - Maximal safe surgical resection (extent of resection prognostic). - Low-risk (age <40 + gross-total resection): observation acceptable. - High-risk (age ≥40 OR subtotal resection): RT + adjuvant PCV (procarbazine + CCNU + vincristine; RTOG-9802 12-yr survival benefit) OR RT + temozolomide (CATNON for grade 3 anaplastic astrocytoma; equivalent for grade 2 in practice). Targeted therapy (paradigm-shifting 2024): - Vorasidenib (oral brain-penetrant pan-IDH1/2 inhibitor): FDA-approved Aug 2024 for IDH-mutant grade 2 astrocytoma / oligodendroglioma after surgery, in patients NOT requiring immediate RT/chemo. INDIGO trial: PFS HR 0.39 vs placebo; transformative for the watch-and-wait window. - Patient selection: residual / progressive non-enhancing disease after surgery; defers RT-induced cognitive toxicity. - Ivosidenib (IDH1-only) explored in earlier LGG trials but vorasidenib is preferred for CNS penetration. Long na...

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