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      Molecular diagnosis and treatment of meningiomas: an expert consensus (2022)

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          Abstract

          Meningiomas are the most common primary intracranial neoplasm with diverse pathological types and complicated clinical manifestations. The fifth edition of the WHO Classification of Tumors of the Central Nervous System (WHO CNS5), published in 2021, introduces major changes that advance the role of molecular diagnostics in meningiomas. To follow the revision of WHO CNS5, this expert consensus statement was formed jointly by the Group of Neuro-Oncology, Society of Neurosurgery, Chinese Medical Association together with neuropathologists and evidence-based experts. The consensus provides reference points to integrate key biomarkers into stratification and clinical decision making for meningioma patients.

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          Practice guideline REgistration for transPAREncy (PREPARE), IPGRP-2022CN234

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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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            The 2021 WHO Classification of Tumors of the Central Nervous System: a summary

            The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS), published in 2021, is the sixth version of the international standard for the classification of brain and spinal cord tumors. Building on the 2016 updated fourth edition and the work of the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy, the 2021 fifth edition introduces major changes that advance the role of molecular diagnostics in CNS tumor classification. At the same time, it remains wedded to other established approaches to tumor diagnosis such as histology and immunohistochemistry. In doing so, the fifth edition establishes some different approaches to both CNS tumor nomenclature and grading and it emphasizes the importance of integrated diagnoses and layered reports. New tumor types and subtypes are introduced, some based on novel diagnostic technologies such as DNA methylome profiling. The present review summarizes the major general changes in the 2021 fifth edition classification and the specific changes in each taxonomic category. It is hoped that this summary provides an overview to facilitate more in-depth exploration of the entire fifth edition of the WHO Classification of Tumors of the Central Nervous System.
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              The 2007 WHO Classification of Tumours of the Central Nervous System

              The fourth edition of the World Health Organization (WHO) classification of tumours of the central nervous system, published in 2007, lists several new entities, including angiocentric glioma, papillary glioneuronal tumour, rosette-forming glioneuronal tumour of the fourth ventricle, papillary tumour of the pineal region, pituicytoma and spindle cell oncocytoma of the adenohypophysis. Histological variants were added if there was evidence of a different age distribution, location, genetic profile or clinical behaviour; these included pilomyxoid astrocytoma, anaplastic medulloblastoma and medulloblastoma with extensive nodularity. The WHO grading scheme and the sections on genetic profiles were updated and the rhabdoid tumour predisposition syndrome was added to the list of familial tumour syndromes typically involving the nervous system. As in the previous, 2000 edition of the WHO ‘Blue Book’, the classification is accompanied by a concise commentary on clinico-pathological characteristics of each tumour type. The 2007 WHO classification is based on the consensus of an international Working Group of 25 pathologists and geneticists, as well as contributions from more than 70 international experts overall, and is presented as the standard for the definition of brain tumours to the clinical oncology and cancer research communities world-wide.
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                Author and article information

                Journal
                Chin Med J (Engl)
                Chin Med J (Engl)
                CM9
                Chinese Medical Journal
                Lippincott Williams & Wilkins (Hagerstown, MD )
                0366-6999
                2542-5641
                20 August 2022
                27 September 2022
                : 135
                : 16
                : 1894-1912
                Affiliations
                [1 ]Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai 200040, China
                [2 ]National Center for Neurological Disorders, Huashan Hospital, Fudan University, Shanghai 200040, China
                [3 ]Shanghai Key Laboratory of Brain Function and Restoration and Neural Regeneration, Shanghai 200040, China
                [4 ]Neurosurgical Institute of Fudan University, Shanghai 200040, China
                [5 ]Shanghai Clinical Medical Center of Neurosurgery, Shanghai 200040, China
                [6 ]Department of Neurosurgery, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
                [7 ]Department of Pathology, Huashan Hospital, Fudan University, Shanghai 200040, China
                [8 ]Department of Neurosurgery, The Affiliated Hospital of Southwest Medical University, Luzhou, Sichuan 646000, China
                [9 ]Department of Neurosurgery, The First Affiliated Hospital of Anhui Medical University, Hefei, Anhui 230022, China
                [10 ]Department of Neurosurgery, The Affiliated Hospital of Inner Mongolia Medical University, Hohhot, Inner Mongolia 750306, China
                [11 ]Department of Neurosurgery, The First Affiliated Hospital of Xinjiang Medical University, Urumqi, Xinjiang 830011, China
                [12 ]Department of Neurosurgery, The First Affiliated Hospital of Nanchang University, Nanchang, Jiangxi 330006, China
                [13 ]Department of Neurosurgery, Shanxi Medical University Shanxi Provincial People's Hospital, Taiyuan, Shanxi 030012, China
                [14 ]Department of Neurosurgery, The Second Xiangya Hospital, Central South University, Changsha, Hunan 410011, China
                [15 ]Department of Neurosurgery, The Second Affiliated Hospital of Soochow University, Soochow, Jiangsu 215004, China
                [16 ]Department of Neurosurgery, Qilu Hospital, Cheeloo College of Medicine and Institute of Brain and Brain-Inspired Science, Shandong University, Jinan, Shandong 250063, China
                [17 ]Department of Neurosurgery, Xiangya Hospital, Central South University, Changsha, Hunan 410008, China
                [18 ]Department of Neurosurgery, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong 510515, China
                [19 ]Department of Neurosurgery, Tangdu Hospital, The Fourth Military Medical University, Xi’an, Shaanxi 710038, China
                [20 ]Department of Neurosurgery, Fujian Medical University Affiliated Union Hospital, Fuzhou, Fujian 350001, China
                [21 ]Department of Neurosurgery, The First Affiliated Hospital of Chongqing Medical University, Chongqing 400042, China
                [22 ]Department of Neurosurgery, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510080, China
                [23 ]Department of Neurosurgery, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu 210029, China
                [24 ]Department of Neurosurgery, Kunming Medical University First Affiliated Hospital, Kunming, Yunnan 650032, China
                [25 ]Department of Neurosurgery, Tianjin Medical University General Hospital, Tianjin 300052, China
                [26 ]Department of Neurosurgery, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China
                [27 ]Department of Neurosurgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
                [28 ]Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China.
                Author notes
                Correspondence to: Prof. Ye Gong, Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai 200040, China E-Mail: gong_ye@ 123456fudan.edu.cn ; Prof. Ping Zhong, Neurosurgical Institute of Fudan University, Huashan Hospital, Fudan University, Shanghai 200040, China E-Mail: zhp228899@ 123456163.com
                Article
                CMJ-2022-1388
                10.1097/CM9.0000000000002391
                9746788
                36179152
                be5a6a29-0486-41b3-b9d8-eaafd53d6a95
                Copyright © 2022 The Chinese Medical Association, produced by Wolters Kluwer, Inc. under the CC-BY-NC-ND license.

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0

                History
                : 19 May 2022
                Categories
                Consensus
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                clinical diagnosis,expert consensus,meningioma,molecular subtyping,therapeutic treatment

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