Gliomatosis cerebri
Encyclopedia
Gliomatosis cerebri 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. Glimatosis Cerebi behaves like a malignant tumor that is very similar to Glioblastoma.
While gliomatosis cerebri can occur at any age, it is generally found in the third and fourth decades of life.
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
. A case of gliomatosis cerebri presenting as rapidly progressive dementia and Parkinson's disease like symptoms was described in an 82 year old woman.
. 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 infiltrative anaplastic astrocytoma
or GBM
.
(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.
Brain tumor
A brain tumor is an intracranial solid neoplasm, a tumor within the brain or the central spinal canal.Brain tumors include all tumors inside the cranium or in the central spinal canal...
. It is commonly characterized by diffuse infiltration of the brain with neoplastic glial cells that affect various areas of the cerebral lobes. Glimatosis Cerebi behaves like a malignant tumor that is very similar to Glioblastoma.
While gliomatosis cerebri can occur at any age, it is generally found in the third and fourth decades of life.
Presentation
It may affect any part of the brainBrain
The brain is the center of the nervous system in all vertebrate and most invertebrate animals—only a few primitive invertebrates such as sponges, jellyfish, sea squirts and starfishes do not have one. It is located in the head, usually close to primary sensory apparatus such as vision, hearing,...
or even the spinal cord
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
, optic nerve
Optic nerve
The optic nerve, also called cranial nerve 2, transmits visual information from the retina to the brain. Derived from the embryonic retinal ganglion cell, a diverticulum located in the diencephalon, the optic nerve doesn't regenerate after transection.-Anatomy:The optic nerve is the second of...
and compact white matter
White matter
White matter is one of the two components of the central nervous system and consists mostly of myelinated axons. White matter tissue of the freshly cut brain appears pinkish white to the naked eye because myelin is composed largely of lipid tissue veined with capillaries. Its white color is due to...
. Clinical manifestations are indefinite, and include headache
Headache
A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
, seizures, visual disturbances, corticospinal tract
Corticospinal tract
The corticospinal or pyramidal tract is a collection of axons that travel between the cerebral cortex of the brain and the spinal cord....
deficits, lethargy, and dementia
Dementia
Dementia is a serious loss of cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging...
. A case of gliomatosis cerebri presenting as rapidly progressive dementia and Parkinson's disease like symptoms was described in an 82 year old woman.
Diagnosis
Before the advent of MRI, diagnosis was generally not established until autopsyAutopsy
An autopsy—also known as a post-mortem examination, necropsy , autopsia cadaverum, or obduction—is a highly specialized surgical procedure that consists of a thorough examination of a corpse to determine the cause and manner of death and to evaluate any disease or injury that may be present...
. 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 infiltrative anaplastic astrocytoma
Astrocytoma
Astrocytomas are a type of neoplasm of the brain. They originate in a particular kind of glial-cells, star-shaped brain cells in the cerebrum called astrocytes. This type of tumor does not usually spread outside the brain and spinal cord and it does not usually affect other organs...
or GBM
Glioblastoma multiforme
Glioblastoma multiforme is the most common and most aggressive malignant primary brain tumor in humans, involving glial cells and accounting for 52% of all functional tissue brain tumor cases and 20% of all intracranial tumors. Despite being the most prevalent form of primary brain tumor, GBMs...
.
Prognosis
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 chemotherapyChemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
(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.