• 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 diffuse, it can be challenging to treat and the prognosis is generally poor. These tumors usually progress like a grade IV glioblastoma multiforme (the most malignant form of brain tumor), aggressively invading normal brain tissue.

    Gliomatosis Cerebri Treatment at Dana-Farber/Boston Children's

    Children and adolescents with gliomatosis cerebri are treated through our 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.

    Our glioma specialists – a team of neuro-oncologists, surgeons, pathologists and radiation oncologists – focus solely on the care of children diagnosed with gliomas. The Glioma Program also offers families the chance to have their child's tumor molecularly profiled (as long as a biopsy can be taken), which may help identify opportunities for targeted treatment.

    What are the gliomatosis cerebri symptoms?

    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 (generally upon awakening), vomiting and seizures
    • Localized symptoms, including weakness or other motor dysfunction and neuroendocrine abnormalities (changes in hormones that affect the nervous system); these tumors invade normal tissue as they grow, and produce symptoms based on their location.
    • 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.

    How is gliomatosis cerebri diagnosed?

    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 (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 compared to normal brain tissue, as well as establish its 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 does a better job of distinguishing between tumors, tumor-related swelling and normal tissue. On an MRI, gliomatosis cerebri will typically appear as a lesion that grows without distinct borders.
    • magnetic resonance spectroscopy (MRS) – a test done along with an MRI. It can detect the presence of organic compounds within sample tissue that can identify the tissue as normal or tumor, and may also be able to tell if the tumor is a glial tumor or if it is of neuronal origin (originating in a neuron, instead of in a brain cell).

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

    What are gliomatosis cerebri treatment options?

    Gliomatosis cerebri 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.
    • experimental chemotherapy delivered along with radiation therapy is actively being investigated as a treatment of gliomatosis cerebri. Several trials evaluating new agents are either underway or have been completed. In addition, there are trials evaluating whether new ways of delivering the traditional drugs might improve responses.

    Unfortunately, surgery is not an option in the treatment of 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?

    Your child may experience side effects from radiation. Radiation often produces inflammation, which can temporarily make symptoms and dysfunctions worse. If your child experiences inflammation, steroids may be prescribed.

    Many specialized brain tumor treatment centers have now added experts in complementary or alternative medicine (CAM). These treatments, including acupuncture/acupressure, therapeutic touch, massage, herbs and dietary recommendations, can also help to control pain and side effects of therapy. Talk to your child's physician about whether CAM might be a viable option of treatment.

    What is the long-term outlook for children with gliomatosis cerebri?

    The prognosis for children with gliomatosis cerebri is generally poor, with a median survival of only 12-24 months. Complete surgical resection is not practical considering the widespread nature of these tumors and the extent of disease, standard chemotherapy has been unsuccessful, and while radiation therapy can stabilize or improve neurological function in some patients, the benefits are temporary and in most cases, tumors will begin to regrow over time.

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

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