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  • Pleomorphic Xanthoastrocytoma (PXA)

    Pleomorphic xanthoastrocytoma (PXA) is a rare, benign brain tumor that likely arises from astrocytes, cells in the nervous system that make up the supportive network for the brain. It is an astrocytoma, which is a type of glioma.

    • Pleomorphic xanthoastrocytoma typically occurs in the cerebral hemisphere (the uppermost sections of the brain) and the leptomeninges, one of the layers covering the brain. Rarely it develops in the spinal cord.
    • Pleomorphic xanthoastrocytomas affect males and females equally; the average age at diagnosis is 12 years.
    • Very rarely, a PXA will transform into a more malignant tumor.

    Pleomorphic Xanthoastrocytoma (PXA) Treatment at Dana-Farber/Boston Children's

    Children and adolescents with pleomorphic xanthoastrocytoma (PXA) 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 symptoms of pleomorphic xanthoastrocytoma (PXA)?

    The most common symptom of PXA at diagnosis is the sudden onset of seizure activity. In fact, nearly 70 percent of children diagnosed with these tumors have seizures. Other, less common symptoms include:

    • Signs of intracranial pressure, such as headache (generally upon awakening), nausea and vomiting
    • Hemiparesis (weakness on one side of the body)
    • Change in behavior

    These tumors, which often occur in children and teenagers, appear to develop spontaneously. 

    How are pleomorphic xanthoastrocytomas 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

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

    What are pleomorphic xanthoastrocytoma (PXA) treatment options?

    The following treatments may be used alone or in combination to treat PXA:

    • Neurosurgery to remove as much of the tumor as possible. If the tumor is completely removed, no other treatment is required other than serial MRIs to monitor for re-growth. A second surgical procedure may be performed if the tumor cannot be completely removed and increases in size, or if it comes back.
    • Radiation therapy may be considered, but does not influence long-term outcomes.
    • Biologic therapy – Researchers at Dana-Farber/Boston Children's have discovered a mutation that occurs in over half of all patients with PXA and there are now new drugs that target this mutation available.

    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

    Recurrences can happen with PXAs. Radiation therapy should be considered for patients with recurrent disease whose tumor has been completely removed. If disease recurs in patients whose tumors have been incompletely removed, a second attempt at surgical removal is usually performed.

    What is the long-term outlook for children with pleomorphic xanthoastrocytoma?

    There is over a 90 percent survival rate for patients whose tumors have been completely removed. After incomplete removal, the long-term survival is still excellent although more therapy is required.

    Survivorship care

    Many brain tumor survivors face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.

    To address the needs of this growing community of brain tumor survivors, Dana-Farber/Boston Children's established the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic. This multi-disciplinary program addresses long-term health and social issues for families and survivors of childhood brain tumors.