Sistema de Salut de Catalunya
Institut Català de la Salut
[Lamoureux AA, Amir-Yazdani P] Department of Neurosciences, University of Montreal, CHU Sainte-Justine, Montreal, Quebec, Canada. [Fisher MJ] Division of Oncology, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania. [Lemelle L] SIREDO Oncology Center (Care, Innovation and Research for Children and AYA with Cancer), PSL Research University, Institut Curie, Paris, France. [Pfaff E] Division of Pediatric Glioma Research, Hopp Children’s Cancer Center (KiTZ), Heidelberg, Germany. National Center for Tumor Diseases (NCT), NCT Heidelberg, A Partnership between DKFZ and Heidelberg University Hospital, Heidelberg, Germany. German Cancer Research Center (DKFZ), Heidelberg, Germany. [Kramm C] Division of Pediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen, Germany. [Llort A] Vall d’Hebron Hospital Universitari, Barcelona, Spain
Vall d'Hebron Barcelona Hospital Campus
2025-03-21T11:23:23Z
2025-03-21T11:23:23Z
2024
2025-02-01
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.
Article
Published version
English
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
American Association for Cancer Research
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/
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