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    BIOMARKER:

    NF1 rearrangement

    i
    Other names: NFNS, NF1, Neurofibromin 1, Neurofibromatosis-Related Protein NF-1, Truncated Neurofibromin 1
    Entrez ID:
    4763
    Related biomarkers:
    Expression
    Mutation
    CNA
    Others
    almost3years
    Malignant Glioma Subset from Actuate 1801 Phase 1/2 Study of 9-ING-41, a Glycogen Synthase Kinase 3 Beta (GSK-3β) Inhibitor, as a Single Agent and Combined with Chemotherapy Refractory (SNO 2021)
    All received initial radiation and temozolomide (18/18), prior salvage therapies included nitrosoureas (15/18), bevacizumab (8/18), TTFields (6/18), checkpoint inhibitor (4/18). Median PFS and OS were 1.9 (0.3-11.1) and 6.0 (1.6-16.6) months, respectively. CONCLUSIONS 9-ING-41 plus/minus lomustine is safe and warrants further study in glioma patients.
    P1/2 data • PD(L)-1 Biomarker • IO biomarker
    |
    EGFR (Epidermal growth factor receptor) • PD-L1 (Programmed death ligand 1) • PTEN (Phosphatase and tensin homolog) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • RB1 (RB Transcriptional Corepressor 1) • NF1 (Neurofibromin 1) • MGMT (6-O-methylguanine-DNA methyltransferase) • TERT (Telomerase Reverse Transcriptase) • PALB2 (Partner and localizer of BRCA2) • LAG3 (Lymphocyte Activating 3) • ATRX (ATRX Chromatin Remodeler)
    |
    TP53 mutation • EGFR mutation • EGFR amplification • PALB2 mutation • NF1 mutation • CDKN2A deletion • CDKN2A mutation • RB1 deletion • TERT mutation • Chr del(10) • NF1 rearrangement
    |
    Avastin (bevacizumab) • temozolomide • lomustine • elraglusib (9-ING-41)
    3years
    Unusual split green-orange signals in USP6 fluorescence in situ hybridization in a malignant peripheral nerve sheath tumor with a novel NF1-SCIMP fusion: a potential diagnostic pitfall. (PubMed, Virchows Arch)
    To the best of our knowledge, this is the first case with a confirmed NF1 gene fusion partner in a peripheral nerve sheath tumor. Notably, rearrangement of the SCIMP may cause a pitfall in the interpretation of USP6 FISH results.
    Journal
    |
    NF1 (Neurofibromin 1)
    |
    NF1 deletion • NF1 rearrangement
    over3years
    [VIRTUAL] Malignant glioma subset from Actuate 1801: A phase 1/2 study of 9-ING-41, a glycogen synthase kinase 3 beta (GSK-3β) inhibitor, as a single agent and combined with chemotherapy, in patients with refractory hematologic malignancies or solid tumors. (ASCO 2021)
    In chemoresistant PDX models of glioblastoma (GBM), 9-ING-41 enhanced the antitumor effect of CCNU and CPT-11...All received first-line radiation and temozolomide (18/18), prior therapies for recurrences included nitrosoureas (15/18), bevacizumab (8/18), TTFields (6/18), immune checkpoint inhibitor (4/18)...Best overall response: 1 minimal response (-43%) after 2 cycles of 9-ING-41 and lomustine . These results show 9-ING-41 alone or in combination is safe and warrants further study in glioma patients.
    Clinical • P1/2 data • PD(L)-1 Biomarker • IO biomarker
    |
    EGFR (Epidermal growth factor receptor) • PD-L1 (Programmed death ligand 1) • PTEN (Phosphatase and tensin homolog) • CDKN2A (Cyclin Dependent Kinase Inhibitor 2A) • RB1 (RB Transcriptional Corepressor 1) • NF1 (Neurofibromin 1) • TERT (Telomerase Reverse Transcriptase) • PALB2 (Partner and localizer of BRCA2) • LAG3 (Lymphocyte Activating 3) • ATRX (ATRX Chromatin Remodeler) • GSK3B (Glycogen Synthase Kinase 3 Beta)
    |
    TP53 mutation • EGFR mutation • EGFR amplification • PALB2 mutation • NF1 mutation • CDKN2A deletion • CDKN2A mutation • RB1 deletion • TERT mutation • TERT promoter mutation • NF1 rearrangement
    |
    Avastin (bevacizumab) • temozolomide • irinotecan • lomustine • elraglusib (9-ING-41)