Show simple item record Lassaletta A Zapotocky M Mistry M Ramaswamy V Honnorat M Krishnatry R Stucklin AG Zhukova N Arnoldo A Ryall S Ling C McKeown, T Loukides J Cruz O de Torres C Ho CY Packer RJ Tatevossian R Qaddoumi I Harreld JH Dalton JD Mulcahy-Levy J Foreman N Karajannis MA Wang SY Snuderl M Rao AN Giannini C Kieran M Ligon KL Garre ML Nozza P Mascelli S Raso A Mueller S Nicolaides T Silva K Perbet R Vasiljevic A Conter CF Frappaz D Leary S Crane C Chan A Ng HK Shi ZF Mao Y Finch E Eisenstat D Wilson B Carret AS Hauser, Péter Sumerauer D Krskova L Larouche V Fleming A Zelcer S Jabado N Rutka JT Dirks P Taylor MD Chen SY Bartels U Huang A Ellison DW Bouffet E Hawkins C Tabori U 2018-12-18T07:57:32Z 2018-12-18T07:57:32Z 2017
dc.identifier 85029215323
dc.identifier.citation pagination=2934-2941; journalVolume=35; journalIssueNumber=25; journalTitle=JOURNAL OF CLINICAL ONCOLOGY;
dc.identifier.uri doi:10.1200/JCO.2016.71.8726
dc.description.abstract Purpose BRAF V600E is a potentially highly targetable mutation detected in a subset of pediatric low-grade gliomas (PLGGs). Its biologic and clinical effect within this diverse group of tumors remains unknown. Patients and Methods A combined clinical and genetic institutional study of patients with PLGGs with long-term follow-up was performed (N = 510). Clinical and treatment data of patients with BRAF V600E mutated PLGG (n = 99) were compared with a large international independent cohort of patients with BRAF V600E mutated-PLGG (n = 180). Results BRAF V600E mutation was detected in 69 of 405 patients (17%) with PLGG across a broad spectrum of histologies and sites, including midline locations, which are not often routinely biopsied in clinical practice. Patients with BRAF V600E PLGG exhibited poor outcomes after chemotherapy and radiation therapies that resulted in a 10-year progression-free survival of 27% (95% CI, 12.1% to 41.9%) and 60.2% (95% CI, 53.3% to 67.1%) for BRAF V600E and wild-type PLGG, respectively (P < .001). Additional multivariable clinical and molecular stratification revealed that the extent of resection and CDKN2A deletion contributed independently to poor outcome in BRAF V600E PLGG. A similar independent role for CDKN2A and resection on outcome were observed in the independent cohort. Quantitative imaging analysis revealed progressive disease and a lack of response to conventional chemotherapy in most patients with BRAF V600E PLGG. Conclusion BRAF V600E PLGG constitutes a distinct entity with poor prognosis when treated with current adjuvant therapy. (C) 2017 by American Society of Clinical Oncology
dc.relation.ispartof urn:issn:0732-183X
dc.title Therapeutic and Prognostic Implications of BRAF V600E in Pediatric Low-Grade Gliomas
dc.type Journal Article 2018-08-31T07:51:30Z
dc.language.rfc3066 en
dc.identifier.mtmt 3341329
dc.identifier.wos 000408568300013
dc.identifier.pubmed 28727518
dc.contributor.institution Semmelweis Egyetem
dc.mtmt.swordnote C.H. and U.T. contributed equally to this work.

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