Molecular Profile Detail

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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 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 Advanced Solid Tumor sensitive Trk Receptor Inhibitor (Pan) Altiratinib Preclinical Actionable In a preclinical study, transformed cells expressing ETV6-NTRK3 demonstrated sensitivity to Altiratinib (DCC-2701) in culture, resulting in inhibition of Ntrk3 phosphorylation and decreased cell proliferation (AACR; Cancer Res 2015;75(15 Suppl):Abstract nr 790). 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 Entrectinib (RXDX-101) 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 Entrectinib (RXDX-101), resulting in inhibition of cell proliferation in culture (PMID: 26939704). 26939704
ETV6-NTRK3 fibrosarcoma predicted - sensitive Trk Receptor Inhibitor (Pan) Entrectinib Phase I Actionable In a Phase I trial, Entrectinib (RXDX-101) 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 salivary gland cancer predicted - sensitive Trk Receptor Inhibitor (Pan) Entrectinib Case Reports/Case Series Actionable In a clinical case study, treatment with Entrectinib (RXDX-101) 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 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 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 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 small intestine neuroendocrine neoplasm predicted - sensitive Trk Receptor Inhibitor (Pan) Entrectinib Case Reports/Case Series Actionable In a clinical study, Entrectinib (RXDX-101) 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 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 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 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 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 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 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 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 gastrointestinal stromal tumor predicted - sensitive Larotrectinib Trk Receptor Inhibitor (Pan) Larotrectinib Phase I 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 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 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 congenital fibrosarcoma not applicable N/A Guideline Diagnostic ETV6-NTRK3 fusions aid the diagnosis of congenital fibrosarcoma (NCCN.org). detail...
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 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
Clinical Trial Phase Therapies Title Recruitment Status
NCT02473497 Expanded access Crizotinib Crizotinib (Xalkori) Expanded Access Protocol For The Treatment Of Adult Or Pediatric Patients Available