• Gliomatosis Cerebri Overview

    Gliomatosis cerebri is a rare, highly aggressive, malignant astrocytic tumor. A variant of glioblastoma multiforme, it 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

    Patients with gliomatosis cerebri are treated at Dana-Farber Boston Children's Cancer and Blood Disorders Center through the Brain Tumor Center. Continue reading to learn more about gliomatosis cerebri or visit the Brain Tumor Center homepage to learn about our expertise with this condition.

    Symptoms & Diagnosis

    Symptoms of gliomatosis cerebri may develop slowly and subtly or they may appear more abruptly. Common symptoms include:

    • Signs of intracranial pressure, 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)
    • Changes in behavior or thought processes

    Gliomatosis cerebri is most commonly diagnosed with diagnostic imaging, including a magnetic resonance spectroscopy, which is done with MRI to detect the presence of organic compounds within sample tissue that can identify normal or tumor tissue and may also be able to tell if the tumor is a glial tumor or of neuronal origin.

    Since these tumors appear to have no primary location and grow aggressively, biopsies can be performed but removal of all of the tumor is not possible.  After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Radiation therapy is the primary treatment for newly diagnosed gliomatosis cerebri. There can be side effects related to the tumor itself or its treatment. Knowing what these side effects are can help physicians prepare for and, in some cases, prevent these symptoms from occurring.

    Progressive or Recurrent Disease

    Clinical trials and experimental therapies are available for children with relapsed, high-grade disease.

    Long-term Outlook

    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.