Pleomorphic Xanthoastrocytoma

  • A pleomorphic xanthoastrocytomas (PXA) is a rare tumor thought to arise from a type of cell of the central nervous system known as a glial cell. These tumors likely originate from a specific type of glial cell known as an astrocyte.

    • Astrocytes make up the supportive network of the brain, and are named for their star-like shape.
    • Pleomorphic xanthoastrocytomas generally arise from the cerebral hemispheres (uppermost sections) of the brain and the leptomeninges (coating of the brain). Rarely do these tumors arise from the spinal column.
    • Pleomorphic xanthoastrocytomas affect males and females equally; the average age at diagnosis is 12 years.
    • Very rarely, these tumors undergo transformation to a more malignant tumor.

    As you read further, you will find general information about pleomorphic xanthoastrocytomas. 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 pleomorphic xanthoastrocytomas

    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 a pleomorphic xanthoastrocytoma?

    Children with these tumors appear to develop them spontaneously, meaning there is no identifiable cause. No genetic component has been discovered.

    What are the symptoms of pleomorphic xanthoastrocytomas?

    The most common symptom at diagnosis is seizure activity.

    Symptoms of pleomorphic xanthoastrocytomas may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.

  • How is a pleomorphic xanthoastrocytoma diagnosed?

    Diagnostic procedures for a pleomorphic xanthoastrocytoma 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. For pleomorphic xanthoastrocytoma, a CT scan of the brain is usually done.
    • 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. For pleomorphic xanthoastrocytoma, MRIs of the brain and spine are usually done.
    • electroencephalogram (EEG) – if your child is experiencing seizures, this procedure records the brain's continuous, electrical activity by means of electrodes attached to the scalp, and helps identify and localize seizure activity to a particular section of the brain.
    • biopsy - a tissue sample from the tumor taken through a needle during a simple surgical procedure performed by a surgeon to confirm the diagnosis
  • What treatments are available for pleomorphic xanthoastrocytomas? 

    Specific treatment for a pleomorphic xanthoastrocytoma may include (alone or in combination):

    Surgery

    The primary treatment is to remove as much as the tumor as possible. If your child's tumor is completely removed, no further treatment other than serial MRIs to monitor for tumor re-growth may be required. If your child's tumor is incompletely removed and then increases in size, or if it comes back, surgery may be performed again.

    Radiation therapy

    May be recommended, although, data suggests that radiation therapy does not influence long-term outcome. This type of therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.

    Are there side effects?

    Your child may have side effects related to both pressure within the brain and to the treatment. An oral steroid such as Dexamethasone may be prescribed to help with symptoms related to increased pressure in the brain, and if your child is experiencing seizures, anti-seizure medications may control them, or in some cases eliminate them altogether.

    What is the expected outcome after treatment for pleomorphic xanthoastrocytoma?

    Fortunately, pleomorphic xanthoastrocytomas are associated with a high rate of cure. Data suggest a long-term survival rate of approximately 90 percent. Greater degree of surgical removal appears to improve long-term survival.

    What about progressive or recurrent disease?

    The recommended treatment for progressive or recurrent pleomorphic xanthoastrocytomas is reattempt at surgical removal.

    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 the New England Phase I Center of the Children's Oncology Group. If your child has progressive or recurrent optic pathway gliomas, she may be eligible for a number of experimental therapies available through these groups, or from one of our independent clinical investigators.

    What is the latest research on pleomorphic xanthoastrocytoma?

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

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