HGG-11. Clinical characteristics and clinical evolution of a large cohort of pediatric patients with primary central nervous system (CNS) tumors and tropomyosin receptor kinase (TRK) fusion.

BACKGROUND: TRK fusions are detected in less than 3% of CNS tumors. Given their rarity, there are limited data on the clinical course of these patients. METHODS: We contacted 166 oncology centers worldwide to retrieve data on patients with TRK fusion-driven CNS tumors. Data extracted included demogr...

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Detalles Bibliográficos
Autores principales: Lamoureux, Audrey-Anne, Fisher, Michael, Lemelle, Lauriane, Pfaff, Elke, Kramm, Christof, De Wilde, Bram, Kazanowska, Bernarda, Hutter, Caroline, Pfister, Stefan M, Sturm, Dominik, Jones, David, Orbach, Daniel, Pierron, Gaëlle, Raskin, Scott, Drilon, Alexander, Diamond, Eli, Harada, Guilherme, Zapotocky, Michal, Ellezam, Benjamin, Weil, Alexander G, Venne, Dominic, Barritault, Marc, Leblond, Pierre, Coltin, Hallie, Hammad, Rawan, Tabori, Uri, Hawkins, Cynthia, Hansford, Jordan R, Meyran, Deborah, Erker, Craig, McFadden, Kathryn, Sato, Mariko, Gottardo, Nicholas G, Dholaria, Hetal, Nørøxe, Dorte Schou, Goto, Hiroaki, Ziegler, David S, Lin, Frank Y, Parsons, Donald Williams, Lindsay, Holly, Wong, Tai-Tong, Liu, Yen-Lin, Wu, Kuo-Sheng, Franson, Andrea Flynn, Hwang, Eugene, Aguilar-Bonilla, Ana, Cheng, Sylvia, Cacciotti, Chantel, Massimino, Maura, Schiavello, Elisabetta, Wood, Paul, Hoffman, Lindsey M, Cappellano, Andréa, Lassaletta, Alvaro, Van Damme, An, Llort, Anna, Gerber, Nicolas U, Ceruso, Mariella Spalato, Bendel, Anne E, Skrypek, Maggie, Hamideh, Dima, Mushtaq, Naureen, Walter, Andrew, Jabado, Nada, Alsahlawi, Aysha, Farmer, Jean-Pierre, Abadi, Christina Coleman, Mueller, Sabine, Mazewski, Claire, Aguilera, Dolly, Robison, Nathan, O’Halloran, Katrina, Abbou, Samuel, Berlanga, Pablo, Geoerger, Birgit, Øra, Ingrid, Moertel, Christopher L, Razis, Evangelia D, Vernadou, Anastasia, Doz, François, Laetsch, Theodore W, Perreault, Sébastien
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
Materias:
High Grade Glioma
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9164744/
http://dx.doi.org/10.1093/neuonc/noac079.226
Descripción
Sumario:BACKGROUND: TRK fusions are detected in less than 3% of CNS tumors. Given their rarity, there are limited data on the clinical course of these patients. METHODS: We contacted 166 oncology centers worldwide to retrieve data on patients with TRK fusion-driven CNS tumors. Data extracted included demographics, histopathology, NTRK gene fusion, treatment modalities and outcomes. Patients less than 18 years of age at diagnosis were included in this analysis. RESULTS: Seventy-three pediatric patients with TRK fusion-driven primary CNS tumors were identified. Median age at diagnosis was 2.4 years (range 0.0–17.8) and 60.2 % were male. NTRK2 gene fusions were found in 37 patients (50.7%), NTRK1 and NTRK3 aberrations were detected in 19 (26.0%) and 17 (23.3%), respectively. Tumor types included 38 high-grade gliomas (HGG; 52.1%), 20 low-grade gliomas (LGG; 27.4%), 4 embryonal tumors (5.5%) and 11 others (15.1%). Median follow-up was 46.5 months (range 3-226). During the course of their disease, a total of 62 (84.9%) patients underwent surgery with a treatment intent, 50 (68.5%) patients received chemotherapy, 35 (47.9%) patients received radiation therapy, while 34 (46.6%) patients received NTRK inhibitors (3 as first line treatment). Twenty-four (32.9%) had no progression including 9 LGG (45%) and 9 HGG (23.6%). At last follow-up, only one (5.6%-18 evaluable) patient with LGG died compared to 11 with HGG (35.5%-31 evaluable). For LGG the median progression-free survival (PFS) after the first line of treatment was 17 months (95% CI: 0.0-35.5) and median overall survival (OS) was not reached. For patients with HGG the median PFS was 30 months (95% CI: 11.9-48.1) and median OS was 182 months (95% CI 20.2-343.8). CONCLUSIONS: We report the largest cohort of pediatric patients with TRK fusion-driven primary CNS tumors. These results will help us to better understand clinical evolution and compare outcomes with ongoing clinical trials.

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