Language

English

Publication Date

2-3-2025

Journal

Clinical Cancer Research

DOI

10.1158/1078-0432.CCR-24-0581

PMID

39625867

PMCID

PMC11788648

PubMedCentral® Posted Date

12-3-2024

PubMedCentral® Full Text Version

Post-print

Abstract

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.

Keywords

Humans, Male, Female, Child, Child, Preschool, Adult, Adolescent, Central Nervous System Neoplasms, Receptor, trkA, Young Adult, Prognosis, Infant, Retrospective Studies, Middle Aged, Glioma, Treatment Outcome, Oncogene Proteins, Fusion, Gene Fusion, Pyrazoles, Pyrimidines, Aged, Receptor, trkB, Protein Kinase Inhibitors

Published Open-Access

yes

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