IDH1 R132 Hotspot-Mutationen treten in ~13-20% des intrahepatischen Cholangiokarzinoms auf und sind F...
Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.
| ID | BMA-IDH1-R132-CHOLANGIO |
|---|---|
| Art | Therapeutische Relevanz |
| Status | reviewed 2026-04-27 | pending_clinical_signoff | actionability review required |
| Erkrankungen | DIS-CHOLANGIOCARCINOMA |
| Quellen | SRC-CIVIC SRC-NCCN-HEPATOBILIARY |
Fakten zur Handlungsfähigkeit
| Biomarker | BIO-IDH-MUTATION |
|---|---|
| Variante | IDH1 R132 hotspot (R132C ~70%, R132L, R132G, R132H, R132S; ~13-20% of intrahepatic cholangiocarcinoma) |
| Erkrankung | DIS-CHOLANGIOCARCINOMA |
| ESCAT-Stufe | IA |
| Empfohlene Kombinationen | ivosidenib monotherapy (2L+ IDH1 R132-mutated cholangio per SRC-NCCN-HEPATOBILIARY) |
| Zusammenfassung der Beweise | 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. |
Anmerkungen
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.
Verwendet von
Anwendungsgebiet
IND-CHOLANGIO-2L-IDH1-IVOSIDENIB- IND-CHOLANGIO-2L-IDH1-IVOSIDENIB