Les mutations des hotspots IDH1 R132 se produisent dans ~13-20% du cholangiocarcinome intrahép...
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| ID | BMA-IDH1-R132-CHOLANGIO |
|---|---|
| Type | Pertinence thérapeutique |
| État | reviewed 2026-04-27 | pending_clinical_signoff | actionability review required |
| Maladies | DIS-CHOLANGIOCARCINOMA |
| Sources | SRC-CIVIC SRC-NCCN-HEPATOBILIARY |
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| Biomarqueur | BIO-IDH-MUTATION |
|---|---|
| Variante | IDH1 R132 hotspot (R132C ~70%, R132L, R132G, R132H, R132S; ~13-20% of intrahepatic cholangiocarcinoma) |
| Maladie | DIS-CHOLANGIOCARCINOMA |
| Niveau ESCAT | IA |
| Combinaisons recommandées | ivosidenib monotherapy (2L+ IDH1 R132-mutated cholangio per SRC-NCCN-HEPATOBILIARY) |
| Résumé des faits | IDH1 R132 hotspot mutations occur in ~13-20% of intrahepatic cholangiocarcinoma and are FDA Level-1 actionable. Ivosidenib was FDA-approved 2021 for previously-treated IDH1-mutated locally advanced/metastatic cholangiocarcinoma based on ClarIDHy (Abou-Alfa Lancet Oncol 2020 — mPFS 2.7 vs 1.4 mo, HR 0.37; OS benefit on rank-preserving structural failure time analysis adjusted for crossover) per SRC-NCCN-HEPATOBILIARY. Comprehensive molecular profiling at diagnosis is recommended to identify IDH1-R132 patients who can be sequenced to ivosidenib in 2L after gemcitabine/cisplatin ± durvalumab (TOPAZ-1) 1L. |
Notes
ESCAT IA / OncoKB Level 1. IDH1 mutations are mutually exclusive with FGFR2 fusions in cholangiocarcinoma; molecular profiling should cover both. Ivosidenib also has on-label use in IDH1-mutated AML (see existing BMAs). Resistance mechanisms: IDH2 isoform switching, receptor tyrosine kinase upregulation. Source-gap: SRC-NCCN-HEPATOBILIARY STUB; SRC-CLARIDHY-ABOU-ALFA-2020 not yet ingested.
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Indication
IND-CHOLANGIO-2L-IDH1-IVOSIDENIB- IND-CHOLANGIO-2L-IDH1-IVOSIDENIB