Pleomorphic Xanthoastrocytoma

What is a pleomorphic astrocytoma?

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 of diagnosis is 12 years. Very rarely, a PXA will transform into a more malignant tumor.

What are the symptoms of pleomorphic xanthoastrocytoma?

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
  • 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 we care for pleomorphic xanthoastrocytoma

Children and adolescents with pleomorphic xanthoastrocytoma (PXA) are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center 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.