Brain_Cancer

What is gliomatosis cerebri?

Gliomatosis cerebri is a highly aggressive, rare form of malignant astrocytic tumor. It most commonly presents as a diffusely infiltrating glial tumor of the cerebral cortex.

What causes gliomatosis cerebri?

There are no known factors or conditions that predispose an individual to the development of gliomatosis cerebri.

What are the symptoms of gliomatosis cerebri?

Gliomatosis cerebri of the cerebral hemispheres can produce non-specific symptoms as a result of increased pressure within the head, as well as more localizing symptoms as a function of specific tumor location, rate of growth and associated inflammation.

The duration of symptoms is variable from a slow, insidious onset to more abrupt presentations. The following are the most common symptoms of gliomatosis cerebri, however each child may experience symptoms differently.

Common symptoms may include:

~headache and lethargy

~symptoms of increased pressure within the brain.

Theresymptomsinclude:headache (generally upon awakening in the morning)

~vomiting

~seizures - can occur with supratentorial high grade gliomas,

but do so less commonly than with low grade lesions

~localized symptoms - these tumors infiltrate normal structures as they grow

and produce symptoms as a function of their location. These can include:

~weakness and other motor dysfunction

~neuroendocrine abnormalities

~changes in behavior or thought processes

How is gliomatosis cerebri classified?

Gliomas are heterogeneous tumors that are classified according to their most aggressive appearing elements. The World Health Organization classification scheme includes 4 grades of glioma. The pathological grade of gliomatosis cerebri is not always established, because only a fraction of these tumors is biopsied. Their clinical course is most consistent with an aggressive form of astrocytic tumor. These tumors are characterized as being of astrocytic origin but having increased numbers of cells (hypercellularity), abnormal cells and nuclei (cytologic and nuclear atypia), increased proliferation of cells (mitoses), increased cell death (necrosis) and increased growth of blood vessels (vascular endothelial proliferation). These are aggressive tumors that infiltrate adjacent normal brain tissue and have a significant tendency to spread outside of the central nervous system.

How is a gliomatosis cerebri diagnosed?

A diagnosis of gliomatosis cerebri is most commonly made from characteristic radiologic studies. They can lack a distinct primary location and their diffusely infiltrating growth makes biopsy a high-risk procedure. Biopsies have been performed when primary masses are identified or when the symptoms and other tests do not seem typical for gliomatosis cerebri.

Diagnostic procedures for gliomatosis cerebri may include:

~physical examination

~computerized tomography scan (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays.A CT scan can assess the density of tumor tissue, as compared to normal brain tissue, as well as establish their mass effect (the effect of an additional space occupying mass within the closed cavity of the skull on the normal brain).

~magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. MRI provides greater anatomical detail than CT scan and can better distinguish between tumor, tumor-related swelling and normal tissue. The typical MRI appearance of gliomatosis cerebri is a lesion that grows without distinct borders, diffusely infiltrating

~magnetic resonance spectroscopy (MRS) - a test done along with MRI at specialized facilities that can detect the presence of particular organic compounds produced by the body's metabolism within sample tissue that can identify tissue as normal or tumor, and may be able to distinguish between glial tumors and tumors of neuronal origin

What are the treatments for a gliomatosis cerebri?

Specific treatment for a gliomatosis cerebri will be determined by your child's physician based on:

~your child's age, overall health, and medical history

~type, location, and size of the tumor

~extent of the disease

~your child's tolerance for specific medications, procedures, or therapies

~how your child's doctors expects the disease to progress

~your opinion or preference

Treatment may include:

~radiation therapy - the primary therapy for newly diagnosed gliomatosis cerebri, radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.

~There is no surgical option in the treatment of these tumors. Experimental chemotherapy delivered concurrent to radiation therapy is actively being investigated in the treatment of diffuse gliomatosis cerebri. Several trials evaluating new agents such as thalidomide and temazolamide are either underway or recently completed. In addition, there are trials evaluating whether improved response to traditional chemotherapeutic agents may be obtained via novel delivery systems to more directly introduce the drugs into the brain. Data on the efficacy of these regimens should be available soon. To date, no chemotherapy regimen has been demonstrated to increase survival in gliomatosis cerebri.

~As with all pediatric cancers, care should be delivered at specialized centers where multidisciplinary teams can provide not only expert diagnostics and experienced medical, surgical and radiation oncologists, but also psychosocial support, neuro-psychological testing and specialized school plans, all delivered in a child- and family-sensitive and friendly environment.

How are side effects managed?

Side effects in the treatment of gliomatosis cerebri can arise from radiation and chemotherapy. Radiation therapy often produces inflammation, which can temporarily exacerbate symptoms and dysfunction. To control this inflammation steroids are sometimes necessary.

Some of the chemotherapy agents are associated with fatigue, diarrhea, constipation and headache. These side effects can be effectively managed under most circumstances with standard medical approaches.

Many specialized brain tumor treatment centers have now added staff who are expert in complementary or alternative medicine. These treatments, including acupuncture/acupressure, therapeutic touch, massage, herbs and dietary recommendations, can also help to control pain and side effects of therapy.

What is the expected outcome after treatment for gliomatosis cerebri?

The prognosis for gliomatosis cerebri remains poor.

What about progressive or recurrent disease?

Clinical trials and experimental therapies are available for patients with relapsed high-grade gliomas at specialized centers. Current trials include novel medications as well as new methods for the delivery of more traditional agents.

What is the latest research on gliomatosis cerebri?

Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat gliomatosis cerebri. For more information on current research, see the Brain Tumor Program.

Gliomatosis Cerebri

Although Gliomatosis Cerebri is very rare and there hasn't been extensive studies on this disease, it is believed to be a pediatric brain tumor.

Below is the best information I have found about Gliomatosis Cerebri. At the bottom of this page you will find links to more information on Gliomatosis Cerebri. You will need to use your browser back button to return to this page.