Gliomatosis cerebri overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

World Health Organization (WHO) defines gliomatosis cerebri (GC) as a distinct nosological entity among other glial tumors of the central nervous system (CNS). Gliomatosis cerebri is classified as a diffusely infiltrating neuroepithelial tumor, which involves at least two cerebral lobes and, occasionally, infratentorial structures or the spinal cord. The brain architecture is commonly preserved, with neurons being spared, and the mass effect is minimal.



Gliomatosis cerebri (infiltrative diffuse astrocytosis) is a rare primary brain tumor. It is commonly characterized by diffuse infiltration of the brain with neoplastic glial cells that affect various areas of the cerebral lobes[1].

It may affect any part of the brain or even the spinal cord, optic nerve and compact white matter. Clinical manifestations are indefinite, and include headache, seizures, visual disturbances, corticospinal tract deficits, lethargy, and dementia.

Before the advent of MRI, diagnosis was generally not established until autopsy. Even with MRI, however, diagnosis is difficult.[2] Typically, gliomatosis cerebri appears as a diffuse, poorly circumscribed, infiltrating non-enhancing lesion that is hyperintense on T2-weighted images and expands the cerebral white matter. It is difficult to distinguish from highly infiltrate anaplastic astrocytoma or GBM.[3] Surgery is not practical considering the extent of the disease, standard chemotherapy (nitrosourea) has been unsuccessful, and while brain irradiation can stabilize or improve neurologic function in some patients, its impact on survival has yet to be proven.[citation needed]

References

  1. http://rad.usuhs.mil/medpix/radpix.html?mode=single&comebackto=mode%3Dgeo_browse&recnum=923
  2. Bendszus M, Warmuth-Metz M, Klein R, Burger R, Schichor C, Tonn JC, Solymosi L (2000). "MR spectroscopy in gliomatosis cerebri". AJNR. American Journal of Neuroradiology. 21 (2): 375–80. PMID 10696026. Retrieved 2012-01-18. Unknown parameter |month= ignored (help)
  3. http://www.urmc.rochester.edu/smd/Rad/neurocases/Neurocase01.htm


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