2026-04-17T02:54:18Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/128222025-10-24T10:16:33Zcom_2072_378070com_2072_378040col_2072_378092

2025-10-24T10:16:33Z urn:hdl:11351/12822 Clinical Characteristics and Outcomes of Central Nervous System Tumors Harboring NTRK Gene Fusions Lamoureux, Audrey-Anne Fisher, Michael Lemelle, Lauriane Pfaff, Elke Amir Yazdani, Pouneh Kramm, Christof Llort, Anna Avaluació de resultats (Assistència sanitària) Proteïnes quinases - Receptors Gliomes - Tractament Sistema nerviós central - Càncer - Tractament Infants Recombinació genètica CHEMICALS AND DRUGS::Amino Acids, Peptides, and Proteins::Proteins::Membrane Proteins::Receptors, Cell Surface::Receptor Protein-Tyrosine Kinases DISEASES::Neoplasms::Neoplasms by Site::Nervous System Neoplasms::Central Nervous System Neoplasms Other subheadings::Other subheadings::/therapy NAMED GROUPS::Persons::Age Groups::Child::Child, Preschool DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Germ Cell and Embryonal::Neuroectodermal Tumors::Neoplasms, Neuroepithelial::Glioma PHENOMENA AND PROCESSES::Genetic Phenomena::Recombination, Genetic::Gene Fusion ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Diagnosis::Prognosis::Treatment Outcome COMPUESTOS QUÍMICOS Y DROGAS::aminoácidos, péptidos y proteínas::proteínas::proteínas de membranas::receptores de superficie celular::receptores proteína-tirosina cinasas ENFERMEDADES::neoplasias::neoplasias por localización::neoplasias del sistema nervioso::neoplasias del sistema nervioso central Otros calificadores::Otros calificadores::/terapia DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño::niño preescolar ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de células germinales y embrionarias::tumores neuroectodérmicos::neoplasias neuroepiteliales::glioma FENÓMENOS Y PROCESOS::fenómenos genéticos::recombinación genética::fusión génica TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::diagnóstico::pronóstico::resultado del tratamiento Clinical characteristics; Central nervous system tumors; NTRK gene fusions Características clínicas; Tumores del sistema nervioso central; Fusiones del gen NTRK Característiques clíniques; Tumors del sistema nerviós central; Fusions del gen NTRK Purpose: Tropomyosin receptor kinase (TRK) fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients. Experimental Design: We conducted an international retrospective cohort study of patients with TRK fusion–driven central nervous system tumors. Results: A total of 119 patients were identified. The median age at the time of diagnosis was 4.5 years. The majority were reported to have a histology consistent with a diagnosis of high-grade glioma (HGG; 57.1%) followed by low-grade glioma (LGG; 27.7%). Pediatric patients had a better prognosis, with a median overall survival of 185.5 months compared with 24.8 months in adults (P < 0.0001). Patients with LGG also had a better outcome when compared with HGG (P = 0.0012). The objective response was 68.8% with larotrectinib compared with 38.1% for nontargeted treatment. Conclusions: Children with LGG had a favorable outcome compared with adult glioma and HGG. TRK inhibitors seem to improve tumor control. 2025-10-24T10:16:33Z 2025-10-24T10:16:33Z 2025-03-21T11:23:23Z 2025-03-21T11:23:23Z 2024 2025-02-01 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://hdl.handle.net/11351/12822 Clinical Cancer Research;31(3) https://doi.org/10.1158/1078-0432.CCR-24-0581 Attribution-NonCommercial-NoDerivatives 4.0 International http://creativecommons.org/licenses/by-nc-nd/4.0/ info:eu-repo/semantics/openAccess American Association for Cancer Research Scientia