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NRG1 fusions occur in ~0.5% of pancreatic ductal adenocarcinoma (PDAC) — a rare but actio...

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

IDBMA-NRG1-FUSION-PDAC
TypeActionability
Statusreviewed 2026-05-03 | pending_clinical_signoff | actionability review required
DiseasesDIS-PDAC
SourcesSRC-ESMO-PANCREATIC-2024 SRC-NCCN-PANCREATIC-2025

Actionability Facts

BiomarkerBIO-NRG1-FUSION
VariantNRG1 gene fusion — any fusion partner (RBPMS-NRG1, SLC3A2-NRG1, PMEPA1-NRG1 among most common in PDAC; detected by RNA-seq)
DiseaseDIS-PDAC
ESCAT tierIIA
Recommended combinationszenocutuzumab 750 mg IV q2w monotherapy (post-gemcitabine-based or post-FOLFIRINOX; CRESTONE regimen)
Evidence summaryNRG1 fusions occur in ~0.5% of pancreatic ductal adenocarcinoma (PDAC) — a rare but actionable subset. Zenocutuzumab (MCLA-128), anti-HER2/HER3 bispecific antibody, FDA accelerated approval (Nov 2024) for NRG1 fusion-positive locally advanced or metastatic NSCLC and pancreatic adenocarcinoma after prior systemic therapy. CRESTONE trial (Schram et al. JCO 2024): NRG1-fusion PDAC cohort — ORR 40%, mDOR 9.1 mo, mPFS 8.7 mo — particularly notable given the ~3 mo mPFS typically seen with 2L-3L chemotherapy in PDAC. NRG1-fusion PDAC is likely KRAS-wild-type in >90% of cases (oncogenic KRAS and NRG1 fusion may be functionally redundant). RNA sequencing required — DNA NGS panels miss most NRG1 fusions.

Notes

ESCAT IIA (accelerated approval based on ORR; confirmatory data pending). NRG1-fusion PDAC is enriched in KRAS-wild-type tumors (testing KRAS early identifies a subpopulation that warrants comprehensive RNA-seq). ORR of 40% in CRESTONE PDAC cohort is particularly impactful given the absence of other effective targeted therapies in 2L-3L PDAC. Testing strategy: if KRAS-wild-type PDAC identified, proceed to RNA-based comprehensive genomic profiling to detect NRG1 (and other rare fusions: NTRK, ALK, RET, FGFR). Zenocutuzumab is generally well tolerated: infusion reactions (premedication required), fatigue, nausea; no grade 3-4 cardiotoxicity seen in CRESTONE.

Used By

No reverse references found in the YAML corpus.