Thalamic H3K27M altered diffuse midline gliomas: Clinicopathological and outcome analysis

• Published in: Clinical neurology and neurosurgery 2024-09, Vol.244, p.108449, Article 108449
• Main Authors: Chaturvedi, Aprajita, Sadashiva, Nishanth, Shukla, Dhaval, Konar, Subhas, Krishna, Uday, Beniwal, Manish, Rao, Shilpa, Santosh, Vani, Saini, Jitender, Vazhayil, Vikas, Prabhuraj, AR, Pruthi, Nupur, Arimappamagan, Arivazhagan
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Adults; Age groups; Biopsy; Brain cancer; Brain Neoplasms - diagnostic imaging; Brain Neoplasms - genetics; Brain Neoplasms - pathology; Brain Neoplasms - therapy; Brain stem; Brain tumors; Cerebrospinal fluid; Chemotherapy; Child; Child, Preschool; Decompression; Diffuse midline gliomas; Female; Glioma; Glioma - genetics; Glioma - pathology; Glioma - therapy; H3K27 altered; Histopathology; Humans; Hydrocephalus; Male; Medical prognosis; Middle Aged; Neuroimaging; Patients; Pediatrics; Radiation therapy; Regression analysis; Retrospective Studies; Survival; Survival analysis; Thalamic glioma; Thalamus; Thalamus - diagnostic imaging; Thalamus - pathology; Tumors; Young Adult
• ISSN: 0303-8467; 1872-6968; 1872-6968
• DOI: 10.1016/j.clineuro.2024.108449

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. 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. 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-