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Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients.

Authors :
Grimaldi, Stéphan
Harlay, Vincent
Appay, Romain
Bequet, Céline
Petrirena, Grégorio
Campello, Chantal
Barrié, Maryline
Autran, Didier
Boissonneau, Sébastien
Graillon, Thomas
Figarella-Branger, Dominique
Nanni, Isabelle
Chinot, Olivier
Tabouret, Emeline
Source :
Journal of Neuro-Oncology; Feb2022, Vol. 156 Issue 3, p615-623, 9p
Publication Year :
2022

Abstract

Background: Adult thalamic gliomas are a rare entity whose management is challenging for physicians. The aim of this study is to describe the characteristics and prognostic factors of thalamic gliomas in adult patients. Methods: We retrospectively analyzed the clinical, neuro-radiological, histological, and molecular characteristics of all cases of adult thalamic glioma in our regional center. Results: We included 38 adult patients. Median age at diagnosis was 56.5 years old (range, 24–80). Median KPS at diagnosis was 70%. Two-thirds of patients presented with tumor necrosis on MRI. Bithalamic lesions were present in four patients. The median volume of enhancement associated with lesions was relatively small (14 mm<superscript>3</superscript>). Two patients had undergone partial surgical resection. All other patients underwent biopsy. Median PFS was 7.1 months (95% CI [3.7–10.5]) and median OS was 15.6 months (95% CI [11.7–19.6]). Among 20 patients with available tumor samples for molecular analyses, only 4 (20%) presented with H3K27M mutation. Patients with H3K27M mutation had longer survival compared to those without. Finally, we identified a long-term survivor population characterized by a younger age, no cognitive impairment, low steroid dose treatment and the presence of H3K27M mutation. Conclusion: Thalamic adult glioma differs from bithalamic glioma in children with regards to its clinical, radiological and molecular profiles. Long-term survival is observed in young patients with limited symptoms and H3K27M mutation. A larger prospective cohort is needed to validate these findings. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
0167594X
Volume :
156
Issue :
3
Database :
Complementary Index
Journal :
Journal of Neuro-Oncology
Publication Type :
Academic Journal
Accession number :
155385211
Full Text :
https://doi.org/10.1007/s11060-022-03943-7