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Gliomatosis Cerebri

Last Updated: March 27, 2020

Figure 1: This diffuse infiltrative glioma was described as gliomatosis cerebri under previous classification systems. Note the diffuse FLAIR (top row) involvement of the right temporal lobe, posterior right frontal lobe, right insula, right basal ganglia and right thalamus. No enhancement is present in this lesion on T1WI after contrast administration.

Basic Description

  • Uncommon, diffusely infiltrating tumor of glial origin involving a large amount of brain parenchyma (previously 3 lobes to meet gliomatosis criteria)

Pathology

  • This tumor type has been deleted from the 2016 WHO Classification
  • A tumor previously described as Gliomatosis Cerebri would now be classified as a diffuse type of astrocytoma, oligodendroglioma, or glioblastoma

Clinical Features

  • Affects all ages (40-50 years most common)
  • No clear gender predilection
  • Altered mental status, dementia, headaches, and seizures are common presenting signs and symptoms
  • Treatment: biopsy and rarely surgical decompression, may require ventricular shunting, variable response to chemoradiation, steroids may be temporarily beneficial
  • Poor prognosis: 1-year survival <50% (median 14 months)
    • Improved survival with lower Ki-67 index, IDH1-positive genetics

Imaging

  • General
    • Diffusely infiltrating, hemispheric white matter mass involving a large area of the brain (previously 3 lobes to meet gliomatosis criteria)
    • Expands and may involve adjacent cortex
    • Basal ganglia, thalami, brainstem, corpus callosum, cerebellum, and spinal cord may be involved
    • Bilateral hemispheric involvement in half of cases
  • CT

    • Asymmetric, poorly-defined, hypoattenuating white matter mass
    • Loss of gray-white matter differentiation
    • Usually nonenhancing on contrast-enhanced CT
  • MRI

    • T1WI: homogeneously iso- to hypointense
    • T2WI: homogeneously hyperintense; hydrocephalus rare
    • FLAIR: homogeneously hyperintense
    • DWI: usually no diffusion restriction
    • T1WI+C: little to no enhancement; areas of enhancement may suggest higher-grade tumor component
    • MR spectroscopy (MRS)/MR perfusion: elevated myoinositol and choline, decreased NAA; high-grade tumor associated with increased relative cerebral blood volume (rCBV)

Imaging Recommendations

  • MRI with contrast, MRS, and MR perfusion may be helpful in equivocal cases

For more information, please see the corresponding chapter in Radiopaedia.

Contributor: Rachel Seltman, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.01.19

References

Arevalo-Perez J, Peck KK, Young RJ. Dynamic contrast-enhanced perfusion MRI and diffusion-weighted imaging in grading of gliomas. J Neuroimaging 2015;25:792–798.

Bendszus M, Warmuth-metz M, Klein R, et al. MR spectroscopy in gliomatosis cerebri. AJNR Am J Neuroradiol. 2000;21: 375-380.

Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114:547.

Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier, 2016.

Shin YM, Chang KH, Han MH, et al. Gliomatosis cerebri: comparison of MR and CT features. AJR Am J Roentgenol. 1993;161: 859-862.

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