Las mutaciones de los hotspots IDH1 R132 se producen en ~13-20% del colangiocarcinoma intrahepático y son F...

Vista determinista de la entidad fuente YAML. La autoridad clínica sigue con los ID de fuente citados y estado de firma del revisor.

ID	`BMA-IDH1-R132-CHOLANGIO`
Tipo	Relevancia terapéutica
Situación	reviewed 2026-04-27 \| pending_clinical_signoff \| actionability review required
Enfermedades	`DIS-CHOLANGIOCARCINOMA`
Fuentes	`SRC-CIVIC` `SRC-NCCN-HEPATOBILIARY`

Hechos relativos a la posibilidad de adoptar medidas

Biomarcador	`BIO-IDH-MUTATION`
Variante	IDH1 R132 hotspot (R132C ~70%, R132L, R132G, R132H, R132S; ~13-20% of intrahepatic cholangiocarcinoma)
Enfermedad	`DIS-CHOLANGIOCARCINOMA`
El nivel de ESCAT	IA
Combinaciones recomendadas	ivosidenib monotherapy (2L+ IDH1 R132-mutated cholangio per SRC-NCCN-HEPATOBILIARY)
Resumen de las pruebas	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.

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.

Utilizado por

Indicación

IND-CHOLANGIO-2L-IDH1-IVOSIDENIB - IND-CHOLANGIO-2L-IDH1-IVOSIDENIB