KIT exon 11 mutation in GIST: imatinib 400 mg/day is standard 1L (B2222, Demetri NEJM 200...

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ID	`BMA-KIT-EXON11-GIST`
Tipo	Relevância terapêutica
Estado	reviewed 2026-04-27 \| pending_clinical_signoff \| actionability review required
Doenças	`DIS-GIST`
Fontes	`SRC-CIVIC` `SRC-IRIS-OBRIEN-2003` `SRC-NCCN-MELANOMA-2025`

Fatos de ação

Biomarcador	`BIO-KIT`
Variante	exon 11 exclusão / inserção ( domínio juxtamembrane — ~50% do GIST)
Doença	`DIS-GIST`
Nível ESCAT	IA
Combinações recomendadas	imatinib 400 mg/day (1L advanced/metastatic), imatinib 400 mg/day adjuvant 3 yr (high-risk resected: ≥3 cm + mitoses ≥5/50 HPF, or rupture, or non-gastric primary), imatinib adjuvant 6 yr (extended for highest-risk per PERSIST-5 / SSG-XXII)
Resumo das provas	KIT exon 11 mutation in GIST: imatinib 400 mg/day is standard 1L (B2222, Demetri NEJM 2002 — ORR 67%; EORTC-62005 / S0033 confirmed long-term benefit). Exon 11 mutants have the longest PFS and OS on imatinib of any GIST genotype. Adjuvant imatinib 3 yr post- resection improves RFS in high-risk disease (SSG-XVIII / ACOSOG-Z9001).

Notas

ESCAT IA. OncoKB Level 1. Source-gap: SRC-NCCN-SARCOMA / SRC-NCCN-GIST-2025 / SRC-B2222-DEMETRI / SRC-EORTC-62005 / SRC-SSG-XVIII not yet ingested — using SRC-NCCN-MELANOMA-2025 placeholder per DIS-GIST source gap. SRC-IRIS-OBRIEN-2003 cited as the imatinib pivotal CML/GIST-era reference. Resistance: secondary KIT exon 13/14 (ATP-binding) or 17/18 (activation loop) mutations emerge during therapy.

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