OncoKB New Data Release - March 30, 2026

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Data Release v7.0 

March 30, 2026

What's New

Updated Therapeutic Implications:

• Promotion of tumor type-specific level of evidence for an alteration

• Level 2: ERBB2 Amplification in small bowel cancer promoted from Level 3A to Level 2 based on Small Bowel Adenocarcinoma NCCN Guideline V1.2026 inclusion of Trastuzumab + Pertuzumab and Trastuzumab + Tucatinib for patients with ERBB2-positive small bowel cancer (PMID: 30857956, 41526345)

• Level 3A sensitivity-associated evidence currently in OncoKB™ for ERBB2 Amplification in small bowel cancer: Trastuzumab Deruxtecan

• Replaced lists of individual mutations with range-based biomarker definitions that group oncogenic and likely oncogenic variants within defined functional domains for select therapeutic associations. Levels of evidence remain unchanged for listed variants

Level	Gene	Previous variant representation	Updated variant nomenclature	Inclusion criteria	Tumor type	Drug(s)
1	ERBB2	L726F/I, T733I, L768S, L755A/P/S/W, I767M, D769H/Y, V773L, G776S/V, V777L/M, V794M, T798M, D808N, G815R, D821N, L841V, V842I, L866M, L869R	Oncogenic missense mutations in the tyrosine kinase domain (TKD)	Oncogenic and likely oncogenic missense mutations in the range 721-975 (the TKD)	Non-Small Cell Lung Cancer	Sevabertinib, Zongertinib
1	ESR1	D538, E380, Y537, L536, L469V, S463P, G442R, F461V	Oncogenic missense mutations in the ligand binding domain (LBD)	Oncogenic and likely oncogenic missense mutations in the range  310-547 (the LBD)	Breast Cancer	Elascetrant
2	POLD1	D402N, E318K, L474P, S478N	Oncogenic missense mutations in the exonuclease domain	Oncogenic and likely oncogenic missense mutations in the range 304-533 (the exonuclease domain)	Colorectal Cancer	Pembrolizumab, Nivolumab, Ipilimumab + Nivolumab, Dostarlimab
					Small Bowel Cancer	Pembrolizumab, Nivolumab, Ipilimumab + Nivolumab, Dostarlimab
2	POLE	A288V, A456P, A463D, A463T, A463V, D275A, D275G, D275V, D368N, D368Y, F367L, F367S, F367V, L424I, L424V, M295R, M444K, P286H, P286L, P286R, P286S, P436H, P436R, P436S, S297F, S297Y, S459F, S459Y, S461L, S461P, S461T, V411L, V411M, V464A, Y458C, Y458H	Oncogenic missense mutations in the exonuclease domain	Oncogenic and likely oncogenic missense mutations in the range 268-471 (the exonuclease domain)	Colorectal Cancer	Pembrolizumab, Nivolumab, Ipilimumab + Nivolumab, Dostarlimab
					Small Bowel Cancer	Pembrolizumab, Nivolumab, Ipilimumab + Nivolumab, Dostarlimab

Gene Curation:

• Addition of 6 new genes:

AVIL GID4 IL2RA NT5E PRMT5 WWOX

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The OncoKB Team