Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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January 17, 2011
Gliomatosis Cerebri
- Gliomatosis cerebri is a rare brain tumor, characterized by diffuse infiltration of the brain with neoplastic cells and preservation of underlying brain architecture.
- It is typically seen in the supratentorial compartment, frequently bilateral, and characterized by involvement of three or more lobes.
- Commonly seen in the third to fifth decades of life, though any age group can be affected. Clinical presentation is usually non-specific.
- 50% mortality: 1 year. 75% mortality: 3 years.
- Key Diagnostic Features: Diffusely infiltrating mass involving three or more lobes without much mass effect is seen. Involvement of the corpus callosum, massa intermedia is often noted. Basal ganglia, the cortex and infratentorial extension is occasionally seen. Minimal to no enhancement is typical. MRS: Elevated mI (myo-inositol), MR perfusion: reduced rCBV.
- DDx: PML, Encephalitis, Leukoaraiosis, Anaplastic astrocytoma
- Treatment: Steroids, Chemotherapy, Radiation therapy
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