Clinical characteristics and outcome of central nervous system tumors harboring NTRK gene fusions
Year: 2025
Type: article
Abstract: PURPOSE 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 CNS tumors. RESULTS 119 patients were identified. The median age at 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 to 24.8 months in adults (p<.0001). Patients with LGG also had a better outcome when compared to HGG (p=0.0012). The objective response was 68.8% with larotrectinib compared to 38.1% for non-targeted treatment. CONCLUSIONS Children with LGG glioma had a favorable outcome compared to adult and HGG. TRK inhibitors appear to improve tumor control.
DOI: 10.5167/uzh-270323
Authors: Lamoureux, Audrey-Anne, Fisher, Michael J, Lemelle, Lauriane, Pfaff, Elke, Amir-Yazdani, Pouneh, Kramm, Christof, De Wilde, Bram, Kazanowska, Bernarda, Hutter, Caroline, Pfister, Stefan M, Sturm, Dominik, Jones, David T W, Orbach, Daniel, Pierron, Gaëlle, Raskin, Scott, Drilon, Alexander, Diamond, Eli L, Harada, Guilherme, Zapotocky, Michal, Zamecnik, Josef, Krskova, Lenka, Ellezam, Benjamin, Weil, Alexander G, Venne, Dominic, Barritault, Marc, Leblond, Pierre, Coltin, Hallie, Hammad, Rawan, Tabori, Uri, Hawkins, Cynthia, Gerber, Nicolas U, et al
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W6948900593
FWCI:
Cites:
Cited by:
Related to: