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.
While gliomatosis cerebri can occur at any age, it is generally found in the third and fourth decades of life.
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
, visual disturbances, corticospinal tract
, and dementia
Before the advent of MRI
, diagnosis was generally not established until autopsy
. Even with MRI, however, diagnosis is difficult. 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
The prognosis for gliomatosis cerebri is generally poor, with a median survival time of only 12 months. 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.