Elsevier

Neurologic Clinics

Volume 38, Issue 1, February 2020, Pages 95-113
Neurologic Clinics

Imaging of Central Nervous System Tumors Based on the 2016 World Health Organization Classification

https://doi.org/10.1016/j.ncl.2019.08.004Get rights and content

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Key points

  • The 2016 World Health Organization Classification of Tumors of the Central Nervous System added a novel classification system for gliomas and medulloblastomas based on phenotypic and molecular characteristics, with the goal to create more homogeneous diagnostic groups to improve patient risk stratification and guide management decisions.

  • Specific radiographic features on anatomic and physiologic MRI have been shown to correlate with certain molecular subgroups in gliomas and medulloblastomas and

Significance

The isocitrate dehydrogenase (IDH) enzymes constitute a group of metabolic enzymes that catalyze the oxidative decarboxylation of isocitrate to α-ketoglutarate in normal cells. Mutations in the IDH gene, as seen in a subgroup of diffuse gliomas and glioblastomas (GBMs), result in conversion of isocitrate to D-2-hydroxyglutarate (D-2HG),7 which, in turn, leads to epigenetic alterations and changes in global genomic methylation patterns that are thought to promote tumorigenesis.8 Approximately

Molecular Subgroups of Medulloblastomas

In addition to retaining the existing histopathologic classification into classic, large cell/anaplastic, desmoplastic/nodular, and extensive nodularity tumors, the 2016 WHO classification distills medulloblastomas into 4 molecular groups: wingless (WNT)-activated, sonic hedgehog (SHH)-activated group 3, and group 4 tumors (see Table 1).66 Notably, each molecular group has distinct prognostic value. Because molecular analyses may not be routinely available at some centers, identification of MRI

Summary

Imaging features on anatomic and physiologic MRI can help distinguish between certain molecular subgroups of diffuse gliomas and medulloblastomas, particularly IDH-mut and IDH-wt gliomas. Radiomics and machine learning–based approaches may augment the ability to distinguish between genetic subgroups by identifying quantitative features from whole-tumor regions of interest. Future studies should focus on prospective validation of these imaging markers in larger and more homogenous patient

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    Disclosures: Dr K.I. Ly has nothing to disclose. Dr P.Y. Wen has received research support from Agios, Astra Zeneca Beigene, Eli Lilly, Genentech/Roche, Karyopharm, Kazia, MediciNova, Merck, Novartis, Oncoceutics, Sanofi-Aventis, and VBI Vaccines. He has served on the advisory board for Abbvie, Agios, Astra Zeneca, Blue Earth Diagnostics, Eli Lilly, Genentech/Roche, Immunomic Therapeutics, Karyopharm, Kiyatec, Puma, Vascular Biogenics, Taiho, Deciphera, VBI Vaccines, and Tocagen; as a speaker for Merck and Prime Oncology; and as an Editor for UpToDate and Elsevier. Dr R.Y. Huang has nothing to disclose.

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