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. Gliomatosis cerebri is a type of astrocytoma, which is a sub-type of glioma. A glioma is a type of brain tumor that originates from glial cells, which support and nourish neurons in the brain. Gliomatosis cerebri is characterized by scattered and widespread tumor cells that cause multiple parts of the brain to enlarge. Because this type of tumor is so diffused, it can be challenging to treat and the prognosis is generally poor. These tumors usually progress like a grade IV glioblastoma multiforme, aggressively invading normal brain tissue.

Gliomatosis cerebri symptoms may develop slowly and subtly or they may appear more abruptly. Each child may experience symptoms differently. Common symptoms include:

• Signs of increased pressure within the brain such as headache
• Localized symptoms, including weakness or other motor dysfunction
• Changes in behavior or thought processes

Gliomatosis cerebri symptoms may resemble those of other conditions or medical problems. Always consult your child's physician for a diagnosis.

Gliomatosis cerebri is most commonly diagnosed with diagnostic imaging. Since often the tumor appears to have no primary location and grows aggressively, biopsies are risky. However, a biopsy may be performed if a primary mass is identified, or if your child’s symptoms and other tests do not seem typical for the condition. Diagnostic procedures for gliomatosis cerebri may include:

• Physical examination
• Computerized tomography scan (CT or CAT scan)
• Magnetic resonance imaging (MRI)
• Magnetic resonance spectroscopy (MRS)

After all tests are completed, doctors will be able to outline the best treatment options.

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 alsopsychosocial support, neuro-psychological testing and specialized school plans, all delivered in a child and family-friendly environment. Gliomatosis cerebri treatment may include radiation therapy or chemotherapy — unfortunately, surgery is not an option.

Children and adolescents with gliomatosis cerebri are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Glioma Program, one of the largest and most experienced pediatric glioma programs in the world, and part of the Dana-Farber/Boston Children’s Brain Tumor Center.