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.
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
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.
Diagnostic procedures for a pleomorphic xanthoastrocytoma may include:
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:
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.
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.
Mark Kieran, MD, PhD, provides a one-hour presentation for parents on latest approaches to treating brain tumors; sponsored by American Brain Tumor Association.