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      EANO guideline on the diagnosis and treatment of vestibular schwannoma

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          Abstract

          The level of evidence to provide treatment recommendations for vestibular schwannoma is low compared with other intracranial neoplasms. Therefore, the vestibular schwannoma task force of the European Association of Neuro-Oncology assessed the data available in the literature and composed a set of recommendations for health care professionals. The radiological diagnosis of vestibular schwannoma is made by magnetic resonance imaging. Histological verification of the diagnosis is not always required. Current treatment options include observation, surgical resection, fractionated radiotherapy, and radiosurgery. The choice of treatment depends on clinical presentation, tumor size, and expertise of the treating center. In small tumors, observation has to be weighed against radiosurgery, in large tumors surgical decompression is mandatory, potentially followed by fractionated radiotherapy or radiosurgery. Except for bevacizumab in neurofibromatosis type 2, there is no role for pharmacotherapy.

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          Most cited references 174

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          The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary.

          The 2016 World Health Organization Classification of Tumors of the Central Nervous System is both a conceptual and practical advance over its 2007 predecessor. For the first time, the WHO classification of CNS tumors uses molecular parameters in addition to histology to define many tumor entities, thus formulating a concept for how CNS tumor diagnoses should be structured in the molecular era. As such, the 2016 CNS WHO presents major restructuring of the diffuse gliomas, medulloblastomas and other embryonal tumors, and incorporates new entities that are defined by both histology and molecular features, including glioblastoma, IDH-wildtype and glioblastoma, IDH-mutant; diffuse midline glioma, H3 K27M-mutant; RELA fusion-positive ependymoma; medulloblastoma, WNT-activated and medulloblastoma, SHH-activated; and embryonal tumour with multilayered rosettes, C19MC-altered. The 2016 edition has added newly recognized neoplasms, and has deleted some entities, variants and patterns that no longer have diagnostic and/or biological relevance. Other notable changes include the addition of brain invasion as a criterion for atypical meningioma and the introduction of a soft tissue-type grading system for the now combined entity of solitary fibrous tumor / hemangiopericytoma-a departure from the manner by which other CNS tumors are graded. Overall, it is hoped that the 2016 CNS WHO will facilitate clinical, experimental and epidemiological studies that will lead to improvements in the lives of patients with brain tumors.
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            DNA methylation-based classification of central nervous system tumours

            Summary Accurate pathological diagnosis is crucial for optimal management of cancer patients. For the ~100 known central nervous system (CNS) tumour entities, standardization of the diagnostic process has been shown to be particularly challenging - with substantial inter-observer variability in the histopathological diagnosis of many tumour types. We herein present the development of a comprehensive approach for DNA methylation-based CNS tumour classification across all entities and age groups, and demonstrate its application in a routine diagnostic setting. We show that availability of this method may have substantial impact on diagnostic precision compared with standard methods, resulting in a change of diagnosis in up to 12% of prospective cases. For broader accessibility we have designed a free online classifier tool (www.molecularneuropathology.org) requiring no additional onsite data processing. Our results provide a blueprint for the generation of machine learning-based tumour classifiers across other cancer entities, with the potential to fundamentally transform tumour pathology.
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              CBTRUS Statistical Report: Primary brain and other central nervous system tumors diagnosed in the United States in 2010-2014.

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                Author and article information

                Contributors
                (View ORCID Profile)
                Journal
                Neuro-Oncology
                Oxford University Press (OUP)
                1522-8517
                1523-5866
                January 2020
                January 11 2020
                October 28 2019
                January 2020
                January 11 2020
                October 28 2019
                : 22
                : 1
                : 31-45
                Affiliations
                [1 ]Center of Neurosurgery, Department of General Neurosurgery, University of Cologne, Cologne, Germany
                [2 ]Department of Neurology, University Hospital and University of Zurich, Zurich, Switzerland
                [3 ]Department of Functional and Stereotactic Neurosurgery and Radiosurgery, Timone University Hospital, Marseille, France
                [4 ]Department of Neurosurgery, Bergen University Hospital and Department of Clinical Medicine, Faculty of Medicine and Dentistry, University of Bergen, Bergen, Norway
                [5 ]Department of Neuropathology, University Hospital Heidelberg, Heidelberg, Germany
                [6 ]Manchester Centre for Genomic Medicine and NW Laboratory Genetics Hub, Manchester University Hospitals NHS Foundation Trust, Manchester, UK
                [7 ]Department of Neurosurgery, Erasmus Hospital, Free University of Brussels, Brussels, Belgium
                [8 ]Department of Neurosurgery, University Hospital San Carlos, Complutense University of Madrid, Madrid, Spain; University Hospital San Carlos, CyberKnife Centre, Genesiscare Madrid, Madrid, Spain
                [9 ]Department of Neuroradiology, IRCCS San Raffaele Scientific Institute and Vita-Salute San Raffaele University, Milan, Italy
                [10 ]Cambridge Skull Base Unit, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
                [11 ]Department of Otolaryngology, Unit of Otology, Auditory implants and Skull Base Surgery, Public Assistance–Paris Hospital, Pitié-Salpêtrière Group Hospital, Paris, France
                [12 ]Unit of Radiotherapy, Neurological Institute Carlo Best, Milan, Italy
                [13 ]Neurology Clinic and National Center for Tumor Diseases, University Hospital Heidelberg, Heidelberg, Germany
                [14 ]Department of Neurosurgery Ludwig-Maximilians University and DKTK partner site, University of Munich, Munich, Germany
                Article
                10.1093/neuonc/noz153
                6954440
                31504802
                © 2019

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