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RT Journal Article
SR Electronic
T1 Molecular GBM versus Histopathological GBM: Radiology-Pathology-Genetic Correlation and the New WHO 2021 Definition of Glioblastoma
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1006
OP 1012
DO 10.3174/ajnr.A8225
VO 45
IS 8
A1 Agarwal, Amit
A1 Edgar, Mark A.
A1 Desai, Amit
A1 Gupta, Vivek
A1 Soni, Neetu
A1 Bathla, Girish
YR 2024
UL http://www.ajnr.org/content/45/8/1006.abstract
AB Given the recent advances in molecular pathogenesis of tumors, with better correlation with tumor behavior and prognosis, major changes were made to the new 2021 World Health Organization (WHO) classification of CNS tumors, including updated criteria for diagnosis of glioblastoma (GBM). Diagnosis of GBM now requires absence of isocitrate dehydrogenase and histone 3 mutations (IDH-wild-type and H3-wild-type) as the basic cornerstone, with elimination of the IDH-mutant category. The requirements for diagnosis were conventionally histopathological, based on the presence of pathognomonic features such as microvascular proliferation and necrosis. However, even if these histologic features are absent, many lower-grade (WHO grade 2/3) diffuse astrocytic gliomas behave clinically similar to GBM (grade 4). The 2021 WHO classification introduced new molecular criteria that can be used to upgrade the diagnosis of such histologically lower-grade, IDH-wild-type, astrocytomas to GBM. The 3 molecular criteria include: concurrent gain of whole chromosome 7 and loss of whole chromosome 10 (+7/–10); telomerase reverse transcriptase promoter mutation; and epidermal growth factor receptor amplification. Given these changes, it is now strongly recommended to have molecular analysis of WHO grade 2/3 diffuse astrocytic, IDH-wild-type, gliomas in adult patients, as identification of any of the above mutations allows for upgrading the tumor to WHO grade 4 (“molecular GBM”) with important prognostic implications. Despite an early stage, there is active ongoing research on the unique MR imaging features of molecular GBM. This paper highlights the differences between “molecular” and “histopathological” GBM, with the aim of providing a basic understanding about these changes.cIMPACT-NOWConsortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy-Not Official WHOCNS52021 WHO classification of CNS tumorsEGFRepidermal growth factor receptorGBMglioblastomaGFAPglial fibrillary acidic proteinGSEAgene set enrichment analysisIDHisocitrate dehydrogenaseMGMTmethylguanine-DNA methyltransferaseMVPmicrovascular proliferationNGSnext-generation sequencingTERTtelomerase reverse transcriptaseTMZtemozolomideWHOWorld Health Organization