Glioblastoma: Changing concepts in the WHO CNS5 classification

Glioblastoma is the most common malignant central nervous system (CNS) tumor in adults. Acute common clinical symptoms include headache, seizure, behavior changes, focal neurological deficits, and signs of increased intracranial pressure. The classic MRI finding of glioblastoma is an irregularly sha...

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Main Authors: Jie Chen, Pengcheng Han, Sonika Dahiya
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2022-01-01
Series:Indian Journal of Pathology and Microbiology
Subjects:
Online Access:http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2022;volume=65;issue=5;spage=24;epage=32;aulast=Chen
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author Jie Chen
Pengcheng Han
Sonika Dahiya
author_facet Jie Chen
Pengcheng Han
Sonika Dahiya
author_sort Jie Chen
collection DOAJ
description Glioblastoma is the most common malignant central nervous system (CNS) tumor in adults. Acute common clinical symptoms include headache, seizure, behavior changes, focal neurological deficits, and signs of increased intracranial pressure. The classic MRI finding of glioblastoma is an irregularly shaped, rim-enhancing or ring-enhancing lesion with a central dark area of necrosis. This constellation of features correlates with microscopic findings of tumor necrosis and microvascular proliferation. Besides these common features, several well-recognized histological subtypes include giant cell glioblastoma, granular cell glioblastoma, gliosarcoma, glioblastoma with a primitive neuronal component, small cell glioblastoma, and epithelioid glioblastoma. While glioblastoma was historically classified as isocitrate dehydrogenase (IDH)-wildtype and IDH-mutant groups, the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy (cIMPACT-NOW) and the fifth edition of the WHO Classification of Tumors of the Central Nervous System clearly updated the nomenclature to reflect glioblastoma to be compatible with wildtype IDH status only. Therefore, glioblastoma is now defined as “a diffuse, astrocytic glioma that is IDH-wildtype and H3-wildtype and has one or more of the following histological or genetic features: microvascular proliferation, necrosis, Telomerase reverse transcriptase promoter mutation, Epidermal growth factor receptor gene amplification, +7/−10 chromosome copy-number changes (CNS WHO grade 4).”
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spelling doaj.art-ef3059281d98464891bd79f167dc72f22022-12-22T00:22:51ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292022-01-01655243210.4103/ijpm.ijpm_1109_21Glioblastoma: Changing concepts in the WHO CNS5 classificationJie ChenPengcheng HanSonika DahiyaGlioblastoma is the most common malignant central nervous system (CNS) tumor in adults. Acute common clinical symptoms include headache, seizure, behavior changes, focal neurological deficits, and signs of increased intracranial pressure. The classic MRI finding of glioblastoma is an irregularly shaped, rim-enhancing or ring-enhancing lesion with a central dark area of necrosis. This constellation of features correlates with microscopic findings of tumor necrosis and microvascular proliferation. Besides these common features, several well-recognized histological subtypes include giant cell glioblastoma, granular cell glioblastoma, gliosarcoma, glioblastoma with a primitive neuronal component, small cell glioblastoma, and epithelioid glioblastoma. While glioblastoma was historically classified as isocitrate dehydrogenase (IDH)-wildtype and IDH-mutant groups, the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy (cIMPACT-NOW) and the fifth edition of the WHO Classification of Tumors of the Central Nervous System clearly updated the nomenclature to reflect glioblastoma to be compatible with wildtype IDH status only. Therefore, glioblastoma is now defined as “a diffuse, astrocytic glioma that is IDH-wildtype and H3-wildtype and has one or more of the following histological or genetic features: microvascular proliferation, necrosis, Telomerase reverse transcriptase promoter mutation, Epidermal growth factor receptor gene amplification, +7/−10 chromosome copy-number changes (CNS WHO grade 4).”http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2022;volume=65;issue=5;spage=24;epage=32;aulast=Chen2021 who classification of tumors of the cns systemcimpact-nowglioblastoma
spellingShingle Jie Chen
Pengcheng Han
Sonika Dahiya
Glioblastoma: Changing concepts in the WHO CNS5 classification
Indian Journal of Pathology and Microbiology
2021 who classification of tumors of the cns system
cimpact-now
glioblastoma
title Glioblastoma: Changing concepts in the WHO CNS5 classification
title_full Glioblastoma: Changing concepts in the WHO CNS5 classification
title_fullStr Glioblastoma: Changing concepts in the WHO CNS5 classification
title_full_unstemmed Glioblastoma: Changing concepts in the WHO CNS5 classification
title_short Glioblastoma: Changing concepts in the WHO CNS5 classification
title_sort glioblastoma changing concepts in the who cns5 classification
topic 2021 who classification of tumors of the cns system
cimpact-now
glioblastoma
url http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2022;volume=65;issue=5;spage=24;epage=32;aulast=Chen
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AT pengchenghan glioblastomachangingconceptsinthewhocns5classification
AT sonikadahiya glioblastomachangingconceptsinthewhocns5classification