Infiltrative gliomas of the thalamus in children: the role of surgery in the era of H3 K27M mutant midline gliomas
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ORIGINAL ARTICLE - TUMOR - GLIOMA
Infiltrative gliomas of the thalamus in children: the role of surgery in the era of H3 K27M mutant midline gliomas Christian Dorfer 1,2 & Thomas Czech 1,2 & Johannes Gojo 2,3 & Arthur Hosmann 1,2 & Andreas Peyrl 2,3 & Amedeo A. Azizi 2,3 & Gregor Kasprian 2,4 & Karin Dieckmann 2,5 & Mariella G. Filbin 6 & Christine Haberler 2,7 & Karl Roessler 1,2 & Irene Slavc 2,3 Received: 5 May 2020 / Accepted: 16 September 2020 # The Author(s) 2020
Abstract 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. Keywords Thalamic tumor . Diffuse midline glioma . Pediatric brain tumor
Abbreviations AA Anaplastic astrocytoma ATRT Atypical teratoid-rhabdoid tumor B Biopsy CNS Central nervous system
CR CTA CTX DOD DTI
Complete remission Computerized tomographic angiography Chemotherapy Dead of disease Diffusion tensor imaging
This article is part of the Topical Collection on Tumor - Glioma No portion of this manuscript has been published or is under consideration for publication elsewhere. * Thomas Czech [email protected] 1
Department of Neurosurgery, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria
2
Comprehensive Cancer Center-CCC CNS Unit, Medical University of Vienna, Vienna, Austria
3
Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
4
Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
5
Department of Radiotherapy, Medical University of Vienna, Vienna, Austria
6
Department of Pediatric Oncology, Dana-Farber Boston Children’s Cancer and Blood Disorders Center, Boston, M
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