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, Marta Perez-Somarriba The Royal Marsden NHS Foundation Trust , London, United Kingdom Hospital Infantil Universitario Niño Jesús , Madrid, Spain Search for other works by this author on: Oxford Academic Anoop Cherungonath The Royal Marsden NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Ajla Wasti University College London Hospital , London, United Kingdom Search for other works by this author on: Oxford Academic Erika Pace The Royal Marsden NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Philip Benjamin The Royal Marsden NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Guillermo Villacampa The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Zita Reisz King’s College Hospital NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Safa Al-Sarraj King’s College Hospital NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Leslie Bridges St George’s Hospital NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Thomas Jacques Great Ormond Street Hospital for Children NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic
, Cinzia Lavarino Pediatric Cancer Center Barcelona , Barcelona, Spain Search for other works by this author on: Oxford Academic Nagore Gene Pediatric Cancer Center Barcelona , Barcelona, Spain Search for other works by this author on: Oxford Academic Rita Pereira The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Yura Grabovska The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Alan Mackay The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Ketty Kessler The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Matthew Clarke The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Vicente Santa-Maria Pediatric Cancer Center Barcelona , Barcelona, Spain Search for other works by this author on: Oxford Academic Ofelia Cruz Pediatric Cancer Center Barcelona , Barcelona, Spain Search for other works by this author on: Oxford Academic Andres Morales La Madrid Pediatric Cancer Center Barcelona , Barcelona, Spain Search for other works by this author on: Oxford Academic Anna Llort Hospital Universitario Vall d´Hebrón , Barcelona, Spain Search for other works by this author on: Oxford Academic Alvaro Lassaletta Hospital Infantil Universitario Niño Jesús , Madrid, Spain Search for other works by this author on: Oxford Academic Felisa Vazquez-Gomez Hospital Infantil Universitario Niño Jesús , Madrid, Spain Search for other works by this author on: Oxford Academic Elena Carceller Hospital Universitario Gregorio Marañón , Madrid, Spain Search for other works by this author on: Oxford Academic Carmen Garrido Hospital Universitario Gregorio Marañón , Madrid, Spain Search for other works by this author on: Oxford Academic Palma Solano-Paez Hospital Universitario Virgen del Rocío , Seville, Spain Search for other works by this author on: Oxford Academic Eduardo Quiroga Hospital Universitario Virgen del Rocío , Seville, Spain Search for other works by this author on: Oxford Academic Bassel Zebian King’s College Hospital NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Samantha Hettige St George’s Hospital NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Julia co*ckle The Royal Marsden NHS Foundation Trust , London, United Kingdom The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Henry Mandeville The Royal Marsden NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Paola Angelini The Royal Marsden NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Kavitha Srivatsa University College London Hospital , London, United Kingdom Search for other works by this author on: Oxford Academic Sucheta Vaidya The Royal Marsden NHS Foundation Trust , London, United Kingdom Search for other works by this author on: Oxford Academic Lynley Marshall The Royal Marsden NHS Foundation Trust , London, United Kingdom The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Chris Jones The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic Fernando Carceller The Royal Marsden NHS Foundation Trust , London, United Kingdom The Institute of Cancer Research , London, United Kingdom Search for other works by this author on: Oxford Academic
Neuro-Oncology, Volume 26, Issue Supplement_4, June 2024, Page 0, https://doi.org/10.1093/neuonc/noae064.321
Published:
18 June 2024
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Marta Perez-Somarriba, Anoop Cherungonath, Ajla Wasti, Erika Pace, Philip Benjamin, Guillermo Villacampa, Zita Reisz, Safa Al-Sarraj, Leslie Bridges, Thomas Jacques, Cinzia Lavarino, Nagore Gene, Rita Pereira, Yura Grabovska, Alan Mackay, Ketty Kessler, Matthew Clarke, Vicente Santa-Maria, Ofelia Cruz, Andres Morales La Madrid, Anna Llort, Alvaro Lassaletta, Felisa Vazquez-Gomez, Elena Carceller, Carmen Garrido, Palma Solano-Paez, Eduardo Quiroga, Bassel Zebian, Samantha Hettige, Julia co*ckle, Henry Mandeville, Paola Angelini, Kavitha Srivatsa, Sucheta Vaidya, Lynley Marshall, Chris Jones, Fernando Carceller, HGG-37. CLINICAL AND MOLECULAR FEATURES OF BITHALAMIC GLIOMAS IN CHILDREN AND ADOLESCENTS: MULTICENTRIC STUDY AND META-ANALYSIS, Neuro-Oncology, Volume 26, Issue Supplement_4, June 2024, Page 0, https://doi.org/10.1093/neuonc/noae064.321
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Abstract
BACKGROUND
Bithalamic gliomas (BTG) are rare brain tumors with dismal prognosis. Optimal management and role of novel therapies have not been established yet. This study aimed to improve our understanding of BTG to guide adapted treatments.
METHODS
Retrospective case series across 6 Pediatric Neuro-Oncology Units (United Kingdom, Spain) of ≤18-year-old patients with BTG between 2000-2022. Clinical, pathological, and molecular data were derived and reviewed. A systematic review of the literature, meta-analysis, and survival analysis were performed.
RESULTS
Overall, 148 BTG cases were evaluated (study=25; literature=123). Of those, 97/148 (65%) had individual survival data. Median overall survival (mOS) (95%CI/range) was 13.2 months (12-16.8/0.6-262.2). mOS stratified by histological grading: 21.0 months (low-grade, n=32) versus 12.0 months (high-grade, n=55); hazard ratio (HR) 0.37 (95%CI 0.20-0.68), p-value=0.001. Molecular analyses (n=70) showed EGFR alterations (56%), TP53 mutations (30%), and H3K27M mutations (23%). mOS of EGFR-altered BTG based on targeted therapy was 13.2 months (no EGFR-inhibitors, n=26) versus 21.6 months (EGFR-inhibitors, n=9); HR 0.36 (95CI% 0.11-1.22). Methylation array data was generated for 4 patients and combined with 10 previously available profiles. Integrated MNP12.8 and mutation information classified 57% as DMG_EGFR and 21% as pedHGG_RTK2B. The remaining cases were classified as DMG_K27M (2/14) or pedHGG_A (1/14). Work is underway to establish a patient-derived EGFR-mutated BTG cell culture and further studies.
CONCLUSIONS
BTG have distinct radiological, and molecular features. EGFR alterations are seen in approximately half of BTG and H3K27M mutations are less frequent than in other midline high-grade gliomas (HGG). Low-grade histology is associated with a survival advantage, although still poor compared to pediatric low-grade gliomas. Therefore, treatment as per HGG guidelines should be considered for all BTG regardless of histological grading. EGFR-altered BTG patients can derive survival benefit from EGFR inhibitors, indicating that these should be considered as part of future treatment protocols.
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© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.
This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.
Issue Section:
Final category: High Grade Glioma
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