CNS tumor
Astrocytic tumors
Gliomatosis cerebri

Author: Eman Abdelzaher, M.D., Ph.D. (see Authors page)

Revised: 29 August 2017, last major update March 2012

Copyright: (c) 2002-2017,, Inc.

PubMed Search: Gliomatosis cerebri [title] central nervous system

Cite this page: Abdelzaher, E. Gliomatosis cerebri. website. Accessed September 22nd, 2018.
Definition / general
  • Rare diffuse glioma (usually astrocytic) with extensive infiltration of a large region of the CNS with involvement of at least three cerebral lobes (WHO definition)
Clinical features
  • MRI and biopsy helpful for diagnosis
  • Any age
  • Often involves thalamus and basal ganglia but may involve entire neuraxis (Radiographics 2003;23:247)
  • May also have a focal mass, often a high grade glioma
  • Variable clinical presentation - usually change in personality and mental status, hemiparesis, ataxia, papilledema
  • Poor prognosis - median survival 12 months
  • More favorable prognosis associated with higher performance status, lower tumor grade (J Neurooncol 2006;76:201, J Neurooncol 2013;112:267)
  • WHO grades vary from II to IV at biopsy but usually progresses to grade IV
Radiology description
  • Best appreciated on T2 weighted or FLAIR MRI as hyperintense diffuse enlargement of involved cerebral regions, without a focal tumor mass
Radiology images

Images hosted on other servers:

MR spectroscopy

Various images

Case reports
  • No effective treatment known
Gross description
  • Autopsy specimens show intact gross anatomy
  • Involved areas are swollen and firm
Gross images

Images hosted on other servers:

Frontal section

Expanded and discolored corpus callosum and fornix

Microscopic (histologic) description
  • Diffuse infiltration of brain parenchyma (with preservation of underlying histoarchitecture) by small, immature glial cells resembling astrocytes, oligodendroglia or undifferentiated cells, with occasional bipolar processes and dense, rod-like irregular nuclei; variable cell density and mitotic activity
  • Epicenter has greater cellularity than surrounding brain
  • Biopsies of margin are difficult to grade
  • Often contains granular calcifications among hypercellular glia
  • Also microcysts and mitotic figures (depending on tumor grade)
  • May have uneven distribution of cellular density that obscures gray white junction or spawns secondary structures of Scherer (subpial and perineuronal neoplastic glia)
Microscopic (histologic) images

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Pons shows
cellular tumor
with markedly
pleomorphic cells

Diffusely infiltrative
glial cells

Optic nerve shows
hypocellular tumor
with mildly
pleomorphic nuclei

Small immature glial cells

GFAP+ cells and fibrillary processes

1: CT shows expansion of white matter in left corona radiata;
2: expanded left cerebral hemisphere with midline shift and compression
of left lateral ventricle; 3: condensation of malignant cells;
4: relative preservation of neurons and architecture

Positive stains
  • GFAP, S100 although oligodendroglioma cells are Leu7+ and S100+ with variable GFAP
Negative stains
Differential diagnosis
  • Gliosis: even distribution of cellular density, contracts instead of expanding near hypercellular glia; usually less pleomorphism, no nuclear hyperchromasia, no nuclear cluster formation, no nuclear molding, no mitotic figures, no calcifications
  • Leptomeningeal gliomatosis: diffuse involvement of leptomeninges by astrocytomas
  • Multifocal glioma: lacks continuity between foci, destructive to normal brain tissue