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As mutações do ponto de acolhimento IDH1 R132 ocorrem em ~13-20% do colangiocarcinoma intra-hepático e são F...

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IDBMA-IDH1-R132-CHOLANGIO
TipoRelevância terapêutica
Estadoreviewed 2026-04-27 | pending_clinical_signoff | actionability review required
DoençasDIS-CHOLANGIOCARCINOMA
FontesSRC-CIVIC SRC-NCCN-HEPATOBILIARY

Fatos de ação

BiomarcadorBIO-IDH-MUTATION
VarianteIDH1 R132 hotspot (R132C ~70%, R132L, R132G, R132H, R132S; ~13-20% of intrahepatic cholangiocarcinoma)
DoençaDIS-CHOLANGIOCARCINOMA
Nível ESCATIA
Combinações recomendadasivosidenib em monoterapia (2L+ IDH1 R132-mutado colangio por SRC-NCCN-HEPATOBILIARY)
Resumo das provasIDH1 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.

Notas

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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