Last Updated: March 27, 2020
- Uncommon, diffusely infiltrating tumor of glial origin involving a large amount of brain parenchyma (previously 3 lobes to meet gliomatosis criteria)
- 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
- 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
- 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
- Asymmetric, poorly-defined, hypoattenuating white matter mass
- Loss of gray-white matter differentiation
- Usually nonenhancing on contrast-enhanced CT
- 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)
- 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
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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