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

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDBMA-KIT-EXON11-GIST
TypeActionability
Statusreviewed 2026-04-27 | pending_clinical_signoff | actionability review required
DiseasesDIS-GIST
SourcesSRC-CIVIC SRC-IRIS-OBRIEN-2003 SRC-NCCN-MELANOMA-2025

Actionability Facts

BiomarkerBIO-KIT
Variantexon 11 deletion / insertion (juxtamembrane domain — ~50% of GIST)
DiseaseDIS-GIST
ESCAT tierIA
Recommended combinationsimatinib 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)
Evidence summaryKIT 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).

Notes

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.

Used By

No reverse references found in the YAML corpus.