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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.

    • Glial means that it originates in the brain’s connective tissue.
    • Astrocyticis another word for glial, and refers to the star-like shape of the astrocyte (a kind of glial cell).

    As you read further below, you’ll find general information about gliomatosis cerebri. If you would like to view summary information about brain tumors first, see the overview on brain tumors.

    How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches gliomatosis cerebri

    We hold a weekly brain tumor clinic for newly diagnosed patients currently receiving treatment. Each time you come for an appointment, you meet with every specialist on your child’s team, from your pediatric neuro-oncologist, neurologist, and neurosurgeon, to your pediatric endocrinologist, psycho-oncologist and School liaison.

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center’s Pediatric Brain Tumor Program offers your child the following services.

    • Access to high-tech resources, like the intra-operative MRI, which allows our pediatric neurosurgeons to visualize the tumor as they operate with MRI scans. This means they can remove as much of the tumor as possible, and sometimes eliminate additional surgeries.
    • Expert neuropathological review, using advanced molecular diagnostic testing, to identify your child’s exact type of tumor. This information helps predict which treatments are more likely to work.
    • Access to unique Phase I clinical trials, from our own investigators, the Children’s Oncology Group and the Pediatric Oncology Experimental Therapeutics Investigators Consortium. Studies offer treatment options beyond standard therapy.
    • Ongoing care from pediatric neurologists familiar with the early symptoms and side effects of brain tumors and their treatments.

    Access to one of the nation’s few dedicated pediatric brain tumor survivorship programs. This weekly clinic offers ongoing care to manage late effects caused by your child’s tumor or the treatment they received.

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center

    44 Binney Street, 3rd Floor
    Boston MA 02115 

    888-PEDI-ONC

  • What causes gliomatosis cerebri?

    There are no known factors or conditions that would make your child more or less likely to develop gliomatosis cerebri.

    What are the symptoms of gliomatosis cerebri?

    Symptoms might come on slowly and subtly, or they might appear more abruptly. Each child may experience symptoms differently, and the most common include:

    • headache and lethargy
    • symptoms of increased pressure within the brain, including:
      • headache (generally upon awakening in the morning)
      • vomiting
      • seizures - can occur with high-grade gliomas in the cerebrum but are less common with low-grade lesions (any abnormal tissue).
    • localized symptoms - these tumors invade normal tissue as they grow, and produce symptoms based on their location. These could include:
    • weakness and other motor dysfunction
    • neuroendocrine (hormones affecting the nervous system) abnormalities
    • changes in behavior or thought processes

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

    How is gliomatosis cerebri classified?

    The World Health Organization classification scheme includes 4 grades of glioma.

    • grade I (benign)
    • grade II (atypical)
    • grade III (anaplastic – refers to lack of structure in the cell)
    • grade IV (sarcomatous – the most serious kind of tumor)

    However, since only a fraction of gliomatosis cerebri tumors are biopsied, it can be difficult to conclusively assign a grade to them.

    That said, they usually progress like grade IV glioblastoma multiforme tumors. These are the most aggressive kind of astrocytic tumor, and they usually have the following characteristics:

    • hypercellularity (increased number of cells)
    • cytologic and nuclear atypia (abnormal cells and nuclei)
    • mitoses (the cells reproduce rapidly)
    • necrosis (the cells die quickly)
    • vascular endothelial proliferation (increased growth of blood vessels)

    These tumors are aggressive, and will invade normal brain tissue. They are also likely to spread outside the central nervous system to other parts of the body.

    What is the latest research on gliomatosis cerebri?

    A variety of chemotherapeutic regimens have been evaluated in the treatment of newly diagnosed high-grade gliomas.

    • While studies in adults have suggested that procarbazine, vincristine and CCNU (PVC) produce modest responses in grade III gliomas, this has not been demonstrated for pediatric cases or for grade IV tumors.
    • Several other regimens have also produced responses, but none has improved survival.
    • Increased doses of chemotherapy in the setting of autologous bone marrow transplant have also not produced notable advantage.

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

  • How is gliomatosis cerebri diagnosed?

    Gliomatosis cerebri is most commonly diagnosed from radiologic studies. 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 indentified, 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).
  • What are the treatments for gliomatosis cerebri?

    Specific treatment for a gliomatosis cerebri 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 (such as thalidomide and temazolamide) are either underway or have been completed. In addition, there are trials evaluating whether new ways of delivering the traditional drugs might improve responses.

    We should know more about the results of these tests soon. To date, no chemotherapy regimen has been shown to increase survival rates.

    Unfortunately, surgery is not an option in the treatment of these tumors.

    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 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 malignant gliomas?

    Our scientists are conducting research studies to help clinicians better understand and treat malignant gliomas. We are a member of the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with relapsed or refractory disease. We are also the New England Phase I Center of the Children's Oncology Group.

    Through these groups, a number of novel therapies are available for children with both newly diagnosed and current brain tumors. Two new protocols include a phase II trial of radiation therapy, cetuximab and irinotecan for children with newly diagnosed malignant glioma and diffuse intrinsic pontine glioma.

    Participation in any clinical trial is completely voluntary: We will take care to fully explain all elements of the treatment plan prior to the start of the trial, and you may remove your child from the medical study at any time.

    Find a clinical trial

    To search for a cancer trial at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, go to:
    http://www.dana-farber.org/Research/Clinical-Trials/Clinical-Trials-by-Diagnosis.aspx?did=21

    To search the NIH’s list of clinical trials taking place around the world, go to: http://www.clinicaltrials.gov/ct2/search

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