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Gliomatosis Cerebri


KEY FACTS

Terminology

  • Diffusely infiltrating, frequently bilateral glial tumor involving at least 3 lobes

  • Infiltrative extent of tumor is out of proportion to histologic and clinical features

Imaging

  • T2 hyperintense infiltrating mass with enlargement of involved structures

    • Typical cerebral hemispheres (75%)

    • Brain architecture enlarged, distorted, but preserved

  • Typically no or minimal enhancement

  • Enhancement may indicate malignant progression or focus of malignant glioma

  • MRS: Increased choline, decreased NAA

Top Differential Diagnoses

  • Arteriolosclerosis

  • Vasculitis

  • Anaplastic astrocytoma

  • Viral encephalitis

  • Demyelination

Pathology

  • Usually WHO grade III; range grade II-IV

  • Shares many features of diffusely infiltrating astrocytoma

  • Rarely, oligodendroglioma is predominant cell type

  • Diagnosis typically made on basis of histology and imaging

Clinical Issues

  • Presenting symptoms: Mental status changes, dementia, headaches, seizures, lethargy

  • Treatment: Biopsy for diagnosis, ± radiation and chemotherapy

  • Peak incidence between 40-50 years, occurs at all ages

  • Relentless progression

  • Survival ranges from weeks to years

    • Median survival ~ 14 months

Axial graphic shows infiltrating tumor involving frontal lobes, insulae, basal ganglia with preservation of the underlying cerebral architecture. Note the focal malignant degeneration
.

Axial T2 MR shows abnormal hyperintensity in the left cerebral peduncle
and left temporal and left frontal lobes with blurring of the corticomedullary junctions
. There is subtle expansion of the involved structures with relative preservation of the underlying architecture, typical of gliomatosis cerebri (GC).

Axial FLAIR MR in a 73-year-old man shows diffuse hyperintensity throughout the supratentorial white matter
, thickening the corpus callosum
but preserving the underlying anatomy. GC may mimic a nonneoplastic white matter disease including arteriolosclerosis.

Axial T1 C+ MR in the same patient shows no enhancement, typical of GC. Enhancement often correlates with focal anaplasia or disease progression. Mild sulcal effacement is present
. These infiltrative tumors may be WHO grade II through IV.

TERMINOLOGY

Abbreviations

  • Gliomatosis cerebri (GC)

Synonyms

  • Gliomatosis, diffuse cerebral gliomatosis

Definitions

  • Diffusely infiltrating, frequently bilateral glial tumor involving at least 3 lobes

  • Infiltrative extent of tumor is out of proportion to histologic and clinical features

IMAGING

General Features

  • Best diagnostic clue

    • T2 hyperintense infiltrating mass with enlargement of involved structures

  • Location

    • Typically hemispheric white matter (WM) involvement (76%), may also involve cortex (19%)

    • 3 lobes, diffuse WM plus

      • –

        Basal ganglia, thalami (75%)

      • –

        Brainstem (52%)

      • –

        Corpus callosum (50%)

      • –

        Cerebellum (29%)

      • –

        Spinal cord (9%)

    • May cross corpus callosum or massa intermedia

  • Morphology

    • Infiltrates, enlarges yet preserves underlying brain architecture

CT Findings

  • NECT

    • Poorly defined, asymmetric low density (often subtle)

    • Loss of gray-white differentiation with expansion and mild mass effect

  • CECT

    • No enhancement typical

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