What Is Childhood Pleomorphic Xanthoastrocytoma?
Pleomorphic xanthoastrocytoma (PXA) is a rare, benign brain tumor that likely arises from astrocytes, cells in the nervous system that make up the brain’s supportive network. It is an astrocytoma, which is a type of glioma.
- Pleomorphic xanthoastrocytoma is a low-grade glioma.
- It 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 xanthoastrocytoma affects males and females equally. The average age at diagnosis is 12 years old.
- Very rarely, a PXA will transform into a more malignant tumor.
At Dana-Farber/Boston Children's Cancer and Blood Disorders Center, our pediatric brain tumor doctors have extensive experience treating children with rare conditions such as pleomorphic xanthoastrocytoma. We use the latest treatments available, including precise surgery and targeted therapy, to provide children with the best possible outcomes. Learn more about our Childhood Glioma Program, part of our comprehensive Brain Tumor Center.
Symptoms of Childhood Pleomorphic Xanthoastrocytoma
The most common symptom of PXA at diagnosis is the sudden onset of seizure activity. 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 We Diagnose Childhood Pleomorphic Xanthoastrocytoma
Your child’s physician will order various diagnostic tests, including imaging studies, an EEG, and a biopsy to confirm the diagnosis. After the tests are complete, your child’s medical team will outline the best treatment options.
How We Treat Childhood Pleomorphic Xanthoastrocytoma
We individualize treatment to every child’s unique situation. Treatment options may include:
- Surgery: The surgeon will remove as much of the tumor as possible. If we’re able to remove the tumor completely, there’s no other treatment necessary. However, we will continue to perform MRIs to monitor for re-growth. If it’s impossible to remove it entirely or it returns, we may perform a second surgical procedure.
- Radiation therapy: We may consider radiation therapy, but we only typically recommend it in the case of disease recurrence.
- Targeted therapy: Targeted therapy, also called precision medicine, works by tailoring treatment to the genetic characteristics of the cancer in an individual child. Researchers at Dana-Farber/Boston Children's have discovered a mutation in over half of all patients with PXA. There are now new drugs available that target this mutation.
We continue to monitor and care for children with pleomorphic xanthoastrocytoma through our extensive pediatric cancer survivorship programs, including the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic for pediatric brain tumor survivors. These services address health and social issues, ranging from motor function evaluation and physical therapy to return-to-school and learning programs.
Long-term Outcomes for Children with Pleomorphic Xanthoastrocytoma
The survival rate is over 90 percent for patients whose tumors have been completely removed. After partial surgical removal, the long-term survival is still excellent, although more therapy is required.
Childhood Pleomorphic Xanthoastrocytoma Treatment Team
Children who come to Dana-Farber/Boston Children’s receive complete care from the world’s top pediatric brain tumor specialists. See a full list of the specialists in our Childhood Brain Tumor Center.