Infiltrative gliomas of the thalamus in children: the role of surgery in the era of H3 K27M mutant midline gliomas

• Published in: Acta neurochirurgica Vol. 163; no. 7; pp. 2025 - 2035
• Main Authors: Dorfer, Christian, Czech, Thomas, Gojo, Johannes, Hosmann, Arthur, Peyrl, Andreas, Azizi, Amedeo A., Kasprian, Gregor, Dieckmann, Karin, Filbin, Mariella G., Haberler, Christine, Roessler, Karl, Slavc, Irene
• Format: Journal Article
• Language: English
• Published: Vienna Springer Vienna 01.07.2021; Springer Nature B.V
• Subjects: Biopsy; Brain cancer; Children; Glioma; Histone H3; Histones; Interventional Radiology; Localization; Medicine; Medicine & Public Health; Minimally Invasive Surgery; Multivariate analysis; Mutants; Neuroimaging; Neurology; Neuroradiology; Neurosurgery; Original - Tumor - Glioma; Original Article - Tumor - Glioma; Patients; Statistical analysis; Surgery; Surgical Orthopedics; Survival; Thalamus; Tumor – Glioma; Diffuse midline glioma; Pediatric brain tumor; Thalamic tumor
• ISSN: 0001-6268; 0942-0940
• DOI: 10.1007/s00701-020-04589-y

Background The role of surgery in the management of pediatric non-pilocytic infiltrative thalamic gliomas needs to be revisited specifically with regard to molecularly defined subtypes. Methods A retrospective review of a consecutive series of children operated on a thalamic tumor between 1992 and May 2018 was performed. Neuroimaging data were reviewed for localization and extent of resection; pathology was re-reviewed according to the current WHO classification, including assessment of histone H3 K27 mutational status. Results Forty-nine patients with a thalamic tumor aged < 18 years at diagnosis were identified. Twenty-five patients (51%) had a non-pilocytic infiltrative glioma, of which the H3 K27M status was available in 22. Fourteen patients were diagnosed as diffuse midline glioma (DMG) H3 K27M mutant . There was no statistically significant difference in survival between patients harboring the H3 K27M mutation and wildtype. Resection (“any resection > 50%” vs “biopsy”) and histological tumor grade (“°II” vs “°III+°IV”) were statistically significant predictors of survival (univariate: p = 0.044 and p = 0.013, respectively). These results remained significant on multivariate analysis (HR 0.371/ p = 0.048, HR 9.433/ p = 0.035). Conclusion We advocate to still consider an attempt at maximal safe resection in the multidisciplinary treatment of unilateral thalamic non-pilocytic gliomas irrespective of their H3 K27-mutational status.