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Profile Name ETV6 - NTRK3
Gene Variant Detail

ETV6 - NTRK3 (gain of function)

Relevant Treatment Approaches IGF-1R Inhibitor Trk Receptor Inhibitor (Pan) Crizotinib Larotrectinib

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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