Thalamic H3K27M altered diffuse midline gliomas: Clinicopathological and outcome analysis.

Autor: Chaturvedi A; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Sadashiva N; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India. Electronic address: nishanth@nimhans.ac.in., Shukla D; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Konar S; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Krishna U; Consultant Radiation Oncology, Apollo Proton Cancer Center, Chennai, India., Beniwal M; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Rao S; Department Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Santosh V; Department Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Saini J; Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Vazhayil V; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Prabhuraj AR; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Pruthi N; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India., Arimappamagan A; Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore 560029, India.
Jazyk: angličtina
Zdroj: Clinical neurology and neurosurgery [Clin Neurol Neurosurg] 2024 Sep; Vol. 244, pp. 108449. Date of Electronic Publication: 2024 Jul 14.
DOI: 10.1016/j.clineuro.2024.108449
Abstrakt: Introduction: Diffuse midline glioma (DMG) is a relatively new entity which was introduced in the fourth edition of the WHO classification of CNS tumours in 2016 and later underwent revision in 2021. It is an infiltrative glioma arising from midline structures, viz., thalamus, spine, and brainstem. Current literature on DMG is based majorly on brainstem lesions, and DMGs arising elsewhere remain unexplored. In our study, we have discussed our experience with thalamic DMGs.
Methodology: This is a retrospective observational study of all patients with histopathologically proven DMG H3K27M altered, arising in the thalamus from 2018 to 2022. Clinical, neuroimaging, and pathology were re-reviewed, and prognostic factors for 3 months, 6 months, and overall survival (OS) were analyzed for all patients.
Results: There were 89 patients- 64 adults and 25 pediatric patients with thalamic DMG. The median age at presentation was 24 years. Raised ICP followed by limb weakness were the most common presenting complaints. Stereotactic biopsy was performed in 64 (71.9 %) patients and surgical decompression in 25 (28.1 %) patients. CSF diversion was required in 53 (59.6 %) patients. Median survival was 8 months in adults and 7 months in pediatric (p-value: 0.51). Raised ICP and TP53 mutation were prognostic factors in pediatric population. Radiotherapy with or without chemotherapy improved survival (p-value- <0.01).
Conclusion: Thalamic DMGs have a poor prognosis which is comparable to brainstem DMGs. Radiotherapy improves survival in these patients. However, the disease remains an enigma and further work delving into its molecular characterization should be encouraged.
Competing Interests: Competing interest All authors certify that they have no affiliations with or involvement in any organisation or entity with any financial or non-financial interest in the subject matter or materials discussed in this manuscript.
(Copyright © 2024 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
Externí odkaz: