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. 

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.

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.

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.