Missing content? – Request curation!
Request curation for specific Genes, variants, or PubMed publications.
Have questions, comments or suggestions? - Let us know!
Email us at : ckbsupport@jax.org
Profile Name | ETV6 - NTRK3 |
Gene Variant Detail | |
Relevant Treatment Approaches | IGF-1R Inhibitor Trk Receptor Inhibitor (Pan) Crizotinib Larotrectinib |
Molecular Profile | Indication/Tumor Type | Response Type | Relevant Treatment Approaches | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
---|---|---|---|---|---|---|---|---|
ETV6 - NTRK3 | salivary gland cancer | predicted - sensitive | Trk Receptor Inhibitor (Pan) | Entrectinib | Case Reports/Case Series | Actionable | In a clinical case study, treatment with Rozlytrek (entrectinib) obtained through a Phase I trial resulted in a durable partial response in a patient with mammary analogue secretory carcinoma of the salivary gland harboring ETV6-NTRK3 (PMID: 26884591; NCT02097810). | 26884591 |
ETV6 - NTRK3 | acute myeloid leukemia | sensitive | Trk Receptor Inhibitor (Pan) | CH7057288 | Preclinical - Cell line xenograft | Actionable | In a preclinical study, CH7057288 inhibited NTRK phosphorylation and downstream signaling and reduced growth of acute myeloid leukemia cells harboring ETV6-NTRK3 in culture, and induced tumor regression in xenograft models (PMID: 30242093). | 30242093 |
ETV6 - NTRK3 | Advanced Solid Tumor | sensitive | IGF-1R Inhibitor | BMS-536924 | Preclinical | Actionable | In a preclinical study, transformed human cells expressing ETV6-NTRK3 were sensitive to BMS-536924, demonstrating a reduction in both cell survival and transformation activity in culture (PMID: 21804605). | 21804605 |
ETV6 - NTRK3 | fibrosarcoma | predicted - sensitive | Trk Receptor Inhibitor (Pan) | Entrectinib | Phase I | Actionable | In a Phase I trial, Rozlytrek (entrectinib) treatment resulted in an objective response in a pediatric patient with infantile fibrosarcoma harboring ETV6-NTRK3 fusion (J Clin Oncol 36, 2018 (suppl; abstr 10536); NCT02650401). | detail... |
ETV6 - NTRK3 | Advanced Solid Tumor | sensitive | Trk Receptor Inhibitor (Pan) | Entrectinib | Preclinical - Cell culture | Actionable | In a preclinical study, transformed cells expressing ETV6-NTRK3 were sensitive to Rozlytrek (entrectinib) as demonstrated by reduced proliferation relative to cells expressing an empty vector in culture (PMID: 26884591). | 26884591 |
ETV6 - NTRK3 | Advanced Solid Tumor | sensitive | Trk Receptor Inhibitor (Pan) | Entrectinib | Preclinical - Cell culture | Actionable | In a preclinical study, transformed cells expressing ETV6-NTRK3 were sensitive to Rozlytrek (entrectinib), resulting in inhibition of cell proliferation in culture (PMID: 26939704). | 26939704 |
ETV6 - NTRK3 | malignant glioma | predicted - sensitive | Trk Receptor Inhibitor (Pan) | Entrectinib | Preclinical - Patient cell culture | Actionable | In a preclinical study, cells from a patient with infant high-grade glioma harboring ETV6-NTRK3 were sensitive to treatment with Rozlytrek (entrectinib) in culture, demonstrating reduced cell viability (PMID: 32238360). | 32238360 |
ETV6 - NTRK3 | Advanced Solid Tumor | sensitive | Larotrectinib Trk Receptor Inhibitor (Pan) | Larotrectinib | Phase II | Actionable | In a combined analysis of three trials, Vitrakvi (larotrectinib) treatment resulted in complete response in 21.4% (6/28) and partial response in 67.9% (19/28) of patients with advanced solid tumors harboring an ETV6-NTRK3 fusion (PMID: 29466156; NCT02122913, NCT02637687, NCT02576431). | 29466156 |
ETV6 - NTRK3 | congenital fibrosarcoma | sensitive | Larotrectinib Trk Receptor Inhibitor (Pan) | Larotrectinib | Case Reports/Case Series | Actionable | In a clinical case study, a patient with congenital fibrosarcoma harboring ETV6-NTRK3 demonstrated tumor regression by 90% when treated with Vitrakvi (larotrectinib) (PMID: 28578312). | 28578312 |
ETV6 - NTRK3 | congenital fibrosarcoma | sensitive | Larotrectinib Trk Receptor Inhibitor (Pan) | Larotrectinib | Phase I | Actionable | In a Phase I clinical trial, treatment with Vitrakvi (larotrectinib) resulted in a partial response in a pediatric patient with infantile fibrosarcoma harboring ETV6-NTRK3 (PMID: 27093299). | 27093299 |
ETV6 - NTRK3 | congenital fibrosarcoma | sensitive | Larotrectinib Trk Receptor Inhibitor (Pan) | Larotrectinib | Phase Ib/II | Actionable | In a Phase I/II trial, Vitrakvi (larotrectinib) treatment resulted in complete response in 33.3% (2/6) and partial response in 66.7% (4/6) of pediatric patients with congenital fibrosarcoma harboring ETV6-NTRK3 fusions (PMID: 29606586; NCT02637687). | 29606586 |
ETV6 - NTRK3 | small intestine neuroendocrine neoplasm | predicted - sensitive | Trk Receptor Inhibitor (Pan) | Entrectinib | Case Reports/Case Series | Actionable | In a clinical study, Rozlytrek (entrectinib) treatment resulted in rapid response and tumor necrosis in a patient with a small intestine neuroendocrine tumor harboring a ETV6-NTRK3 fusion (PMID: 29118225; NCT02568267). | 29118225 |
ETV6 - NTRK3 | malignant glioma | predicted - sensitive | Milciclib | Preclinical - Patient cell culture | Actionable | In a preclinical study, cells from a patient with infant high-grade glioma harboring ETV6-NTRK3 were sensitive to treatment with Milciclib (PHA-848125AC) in culture, demonstrating reduced cell viability (PMID: 32238360). | 32238360 | |
ETV6 - NTRK3 | Advanced Solid Tumor | sensitive | Trk Receptor Inhibitor (Pan) | DS6051b | Preclinical - Cell culture | Actionable | In a preclinical study, transformed cells expressing ETV6-NTRK3 were sensitive to treatment with DS6051b in culture, demonstrating cell growth inhibition (PMID: 31399568). | 31399568 |
ETV6 - NTRK3 | acute myeloid leukemia | sensitive | Larotrectinib Trk Receptor Inhibitor (Pan) | Larotrectinib | Preclinical | Actionable | In a preclinical study, Vitrakvi (larotrectinib) inhibited cell growth and downstream signaling in acute myeloid leukemia cells harboring ETV6-NTRK3 in culture and xenografts (PMID: 26216294). | 26216294 |
ETV6 - NTRK3 | Advanced Solid Tumor | sensitive | Trk Receptor Inhibitor (Pan) | Altiratinib | Preclinical | Actionable | In a preclinical study, Altiratinib (DCC-2701) inhibited Ntrk3 downstream signaling and proliferation of transformed cells expressing ETV6-NTRK3 in culture (PMID: 33328556). | 33328556 |
ETV6 - NTRK3 | B-lymphoblastic leukemia/lymphoma with BCR-ABL1 | predicted - sensitive | Larotrectinib Trk Receptor Inhibitor (Pan) | Larotrectinib | Case Reports/Case Series | Actionable | In a clinical case study, Vitrakvi (larotrectinib) treatment resulted in significant suppression of leukemic cells and clinical benefit in a patient with relapsed Philadelphia chromosome (Ph)-like acute lymphoblastic leukemia associated with an expansion of a subclone harboring ETV6-NTRK3 fusion (PMID: 31905241). | 31905241 |
ETV6 - NTRK3 | melanoma | sensitive | Trk Receptor Inhibitor (Pan) | DS-6051a | Preclinical - Cell culture | Actionable | In a preclinical study, DS-6051a inhibited MAPK, PI3K, and PLCgamma-1 signaling in transformed melanocytes expressing ETV6-NTRK3 in culture (PMID: 27477320). | 27477320 |
ETV6 - NTRK3 | breast cancer | sensitive | IGF-1R Inhibitor | BMS-754807 | Preclinical - Cell line xenograft | Actionable | In a preclinical study, BMS-754807 inhibited tumor growth in cell line xenograft models of breast cancer cells overexpressing ETV6-NTRK3 (PMID: 21148487). | 21148487 |
ETV6 - NTRK3 | pancreatic cancer | predicted - sensitive | Trk Receptor Inhibitor (Pan) | PLX7486 | Case Reports/Case Series | Actionable | In a clinical case study, a patient with pancreatic cancer harboring ETV6-NTRK3 demonstrated a prolonged response when treated with PLX7486 (PMID: 31406350). | 31406350 |
ETV6 - NTRK3 | malignant glioma | predicted - sensitive | Crizotinib | Crizotinib | Preclinical - Patient cell culture | Actionable | In a preclinical study, cells from a patient with infant high-grade glioma harboring ETV6-NTRK3 were sensitive to treatment with Xalkori (crizotinib) in culture, demonstrating reduced cell viability (PMID: 32238360). | 32238360 |
ETV6 - NTRK3 | head and neck squamous cell carcinoma | sensitive | Trk Receptor Inhibitor (Pan) | Merestinib | Preclinical - Pdx | Actionable | In a preclinical study, a head and neck squamous cell carcinoma patient-derived xenograft (PDX) model harboring ETV6-NTRK3 was sensitive to treatment with Merestinib (LY2801653) compared to vehicle, demonstrating decreased tumor growth (PMID: 29568395). | 29568395 |
ETV6 - NTRK3 | acute myeloid leukemia | sensitive | Crizotinib | Crizotinib | Preclinical - Cell line xenograft | Actionable | In a preclinical study, Xalkori (crizotinib) inhibited ETV6-NTRK3 activity, reduced cell growth in a human acute myeloid leukemia cell line harboring ETV6-NTRK3, and had anti-tumor activity in xenograft models (PMID: 23811600). | 23811600 |
ETV6 - NTRK3 | glioblastoma | predicted - sensitive | Larotrectinib Trk Receptor Inhibitor (Pan) | Larotrectinib | Case Reports/Case Series | Actionable | In a clinical case study, an 18-month-old patient with glioblastoma harboring ETV6-NTRK3 was treated with Viktravi (larotrectinib) and demonstrated tumor regression after 8 weeks and remained clinically stable after 6 months of therapy (PMID: 32923892). | 32923892 |
ETV6 - NTRK3 | gastrointestinal stromal tumor | predicted - sensitive | Larotrectinib Trk Receptor Inhibitor (Pan) | Larotrectinib | Case Reports/Case Series | Actionable | In a Phase I trial, Vitrakvi (larotrectinib) treatment resulted in complete response in 1 and partial response in 2 patients with gastrointestinal stromal tumor harboring ETV6-NTRK3 fusions (PMID: 29466156, Annals of Oncology, Volume 29, Issue suppl_5; NCT02122913, NCT02637687, and NCT02576431). | 29466156 detail... |
ETV6 - NTRK3 | Advanced Solid Tumor | sensitive | Trk Receptor Inhibitor (Pan) | Repotrectinib | Preclinical - Cell culture | Actionable | In a preclinical study, Repotrectinib (TPX-0005) inhibited proliferation of transformed cells expressing ETV6-NTRK3 in culture (PMID: 30093503). | 30093503 |
ETV6 - NTRK3 | Advanced Solid Tumor | sensitive | Trk Receptor Inhibitor (Pan) | Lestaurtinib | Preclinical | Actionable | In a preclinical study, transformed cells expressing the fusion, ETV6-NTRK3, were treated with Lestaurtinib (CEP-701), which resulted in inhibition of ETV6-NTRK3 autophosphorylation and restoration of Tgf-b1 signaling (PMID: 16258068). | 16258068 |
ETV6 - NTRK3 | salivary gland cancer | predicted - sensitive | Crizotinib | Crizotinib | Case Reports/Case Series | Actionable | In a clinical case study, Xalkori (crizotinib) treatment resulted in a brief stable disease in a patient with mammary analogue secretory carcinoma of the salivary gland harboring ETV6-NTRK3, before disease progression at 18 weeks (PMID: 26884591). | 26884591 |
ETV6 - NTRK3 | cancer | sensitive | Midostaurin | Preclinical | Actionable | In a preclinical study, the broad spectrum kinase inhibitor midostaurin inhibited growth of cells dependent on the fusion protein ETV6-NTRK3, reducing ETV6-NTRK3 kinase activity and downstream signaling and inducing apoptosis (PMID: 23131561). | 23131561 | |
ETV6 - NTRK3 | congenital fibrosarcoma | not applicable | N/A | Guideline | Diagnostic | ETV6-NTRK3 fusions aid the diagnosis of congenital fibrosarcoma (NCCN.org). | detail... |
Clinical Trial | Phase | Therapies | Title | Recruitment Status | Covered Countries | Other Countries |
---|---|---|---|---|---|---|
NCT02473497 | Expanded access | Crizotinib | Crizotinib (Xalkori) Expanded Access Protocol For The Treatment Of Adult Or Pediatric Patients | Available | USA | 0 |