Pleomorphic xanthoastrocytoma (PXA) is a rare, benign tumor that likely arises from astrocytes, cells in the nervous system that make up the supportive network for the brain. They typically occur in the cerebral hemispheres (the uppermost sections of the brain) and the leptomeninges, one of the layers covering the brain. They rarely develop in the spinal cord.
Patients with PXA are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Brain Tumor Center. Continue reading to learn more about PXA or visit the Brain Tumor Center homepage to learn about our expertise with this condition.
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:
These tumors, which often occur in children and teenagers, appear to develop spontaneously. Very rarely do they transform into malignant tumors.
In addition to a complete physical exam, doctors diagnose PXA with:
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:
Doctors at Dana-Farber 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.
Mark Kieran, MD, PhD, provides a one-hour presentation for parents on latest approaches to treating brain tumors; sponsored by American Brain Tumor Association.