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, PathologyOutlines.com, Inc.

PubMed Search: Gliomatosis cerebri [title] central nervous system

Cite this page: Abdelzaher, E. Gliomatosis cerebri. PathologyOutlines.com website. http://pathologyoutlines.com/topic/cnstumorgliomatosiscerebri.html. Accessed October 22nd, 2017.
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)
Grading
  • 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
Treatment
  • 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

Images hosted on other servers:

Pons shows
cellular tumor
with markedly
pleomorphic cells

Diffusely infiltrative
moderately
pleomorphic
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