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EPID-10. CNSONTRK STUDY: CLINICAL CHARACTERISTICS AND OUTCOME OF CENTRAL NERVOUS SYSTEM TUMORS HARBORING NTRK GENE FUSIONS

Lamoureux, Audrey-Anne ; Fisher, Michael ; Lemelle, Lauriane ; Pfaff, Elke ; De Wilde, Bram ; Pfister, Stefan ; Sturm, Dominik ; Orbach, Daniel ; Raskin, Scott ; Drilon, Alexander ; Zapotocky, Michal ; Barritault, Marc ; Leblond, Pierre ; Tabori, Uri ; Hawkins, Cynthia ; Hansford, Jordan ; Erker, Craig ; Gottardo, Nicholas ; Nørøxe, Dorte ; Goto, Hiroaki ; Ziegler, David ; Liu, Yen-Lin ; Hwang, Eugene ; Aguilar-Bonilla, Ana ; Cheng, Sylvia ; Cacciotti, Chantel ; Massimino, Maura ; Wood, Paul ; Hoffman, Lindsey ; Lassaletta, Alvaro ; Gerber, Nicolas ; Ceruso, Mariella Spalato ; Bendel, Anne ; Abadi, Christina Coleman ; Mueller, Sabine ; Aguilera, Dolly ; Robison, Nathan ; O’Halloran, Katrina ; Abbou, Samuel ; Geoerger, Birgit ; Øra, Ingrid ; Moertel, Christopher ; Razis, Evangelia ; Seizeur, Romuald ; Clarke, Matthew ; Doz, François ; Laetsch, Theodore ; Perreault, Sébastien ; Ellezam, Benjamin ; Alves, Mélanie

Neuro-oncology (Charlottesville, Va.), 2023-11, Vol.25 (Supplement_5), p.v117-v117 [Periódico revisado por pares]

US: Oxford University Press

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EPID-10. CNSONTRK STUDY: CLINICAL CHARACTERISTICS AND OUTCOME OF CENTRAL NERVOUS SYSTEM TUMORS HARBORING NTRK GENE FUSIONS
Autor: Lamoureux, Audrey-Anne ; Fisher, Michael ; Lemelle, Lauriane ; Pfaff, Elke ; De Wilde, Bram ; Pfister, Stefan ; Sturm, Dominik ; Orbach, Daniel ; Raskin, Scott ; Drilon, Alexander ; Zapotocky, Michal ; Barritault, Marc ; Leblond, Pierre ; Tabori, Uri ; Hawkins, Cynthia ; Hansford, Jordan ; Erker, Craig ; Gottardo, Nicholas ; Nørøxe, Dorte ; Goto, Hiroaki ; Ziegler, David ; Liu, Yen-Lin ; Hwang, Eugene ; Aguilar-Bonilla, Ana ; Cheng, Sylvia ; Cacciotti, Chantel ; Massimino, Maura ; Wood, Paul ; Hoffman, Lindsey ; Lassaletta, Alvaro ; Gerber, Nicolas ; Ceruso, Mariella Spalato ; Bendel, Anne ; Abadi, Christina Coleman ; Mueller, Sabine ; Aguilera, Dolly ; Robison, Nathan ; O’Halloran, Katrina ; Abbou, Samuel ; Geoerger, Birgit ; Øra, Ingrid ; Moertel, Christopher ; Razis, Evangelia ; Seizeur, Romuald ; Clarke, Matthew ; Doz, François ; Laetsch, Theodore ; Perreault, Sébastien ; Ellezam, Benjamin ; Alves, Mélanie
É parte de: Neuro-oncology (Charlottesville, Va.), 2023-11, Vol.25 (Supplement_5), p.v117-v117
Descrição: Abstract BACKGROUND TRK fusions are detected in less than 2% of central nervous system (CNS) tumors. There are limited data on the clinical course of affected patients. METHODS We conducted an international retrospective cohort study of patients with TRK fusion-driven CNS tumors. Data extracted included demographics, histopathology, TRK gene fusion, treatment modalities and outcomes. The growth modulation index (GMI) was calculated as a ratio of time to progression with TRK inhibitors to time to progression with the prior line of therapy. RESULTS 119 patients with TRK fusion-driven primary CNS tumors were identified. Median age at diagnosis was 4.5 years (range 0.0–78.3); 101 (85.6%) were < 18 and 45.3% were < 3 years of age. The most frequent tumor location was hemispheric (63.8%). Tumor types included 68 high-grade gliomas (HGG; 57.1%), 33 low-grade gliomas (LGG; 27.7%), 5 embryonal tumors (4.2%) and 13 others (10.9%). Median follow-up was 38.5 months (range 0.03–229.3). Pediatric patients had a better prognosis with a median OS of 185.5 months compared to 24.8 months in adults (p< .0001). Patients with LGG also had a better outcome with median OS that was not reached for LGG compared to 99.5 months for HGG and 38.5 months for embryonal tumors (p=0.0012). Out of 16 evaluable treatment regimens with larotrectinib an objective response was reported in 11 (11/16 (68.8%)-4 CR, 5 PR and 2 MR) compared to 38.1% for non-targeted treatment regimens (24/63 (38.1%)-10 CR, 13 PR and 1 MR). The GMI was 2.11 for pediatric patients treated with larotrectinib. CONCLUSIONS We report a large cohort of patients with TRK fusion-driven primary CNS tumors. Children with LGG glioma had a favorable outcome compared to adult and HGG. Larotrectinib appears to be associated with a better response rate and longer duration of response compared to non-targeted therapy.
Editor: US: Oxford University Press
Idioma: Inglês

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EPID-10. CNSONTRK STUDY: CLINICAL CHARACTERISTICS AND OUTCOME OF CENTRAL NERVOUS SYSTEM TUMORS HARBORING NTRK GENE FUSIONS

US: Oxford University Press

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