Gliomatosis cerebri
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Gliomatosis cerebri (infiltrative diffuse sstrocytosis) is a rare primary brain tumor characterized by diffuse infiltration of the brain with neoplastic glial cells that typically involve multiple brain areas. The morphology of tumor cells is diverse, taking on the appearance of astrocytes, oligodendrocytes, or Schwann cells with variable mitotic activity. It represents an extreme form of diffusely infiltrating glioma. It is difficult to distinguish gliomatosis cerebri from highly infiltrate anaplastic astrocytoma or GBM.
Gliomatosis cerebri can occur at any age but is usually 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 non-specific, and include headache, seizures, visual disturbances, corticospinal tract deficits, lethargy, and dementia.
Before the advent of MRI, diagnosis was generally not established until autopsy. On MRI, it typically appears as a diffuse, poorly circumscribed, infiltrating non-enhancing lesion that is hyperintense on T2-weighted images and expands the cerebral white matter.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 although brain irradiation can stabilize or improve neurologic function in some patients, its impact on survival has yet to be proven.
