Pleomorphic Xanthoastrocytoma in Children

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A pleomorphic xanthoastrocytomas (PXA) is a rare tumor thought to arise from a type of cell of the central nervous system known as a glial cell. These tumors likely originate from a specific type of glial cell known as an astrocyte.

  • Astrocytes make up the supportive network of the brain, and are named for their star-like shape.
  • Pleomorphic xanthoastrocytomas generally arise from the cerebral hemispheres (uppermost sections) of the brain and the leptomeninges (coating of the brain). Rarely do these tumors arise from the spinal column.
  • Pleomorphic xanthoastrocytomas affect males and females equally; the average age at diagnosis is 12 years.
  • Very rarely, these tumors undergo transformation to a more malignant tumor.

As you read further, you will find general information about pleomorphic xanthoastrocytomas. If you would like to view summary information about brain tumors first, see the overview on brain tumors.

How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches pleomorphic xanthoastrocytomas

We hold a weekly brain tumor clinic for newly diagnosed patients currently receiving treatment. Each time you come for an appointment, you meet with every specialist on your child’s team, from your pediatric neuro-oncologist, neurologist, and neurosurgeon, to your pediatric endocrinologist, psycho-oncologist and School liaison.

Dana-Farber/Boston Children’s Cancer and Blood Disorders Center’s Pediatric Brain Tumor Program offers your child the following services.

  • Access to high-tech resources, like the intra-operative MRI, which allows our pediatric neurosurgeons to visualize the tumor as they operate with MRI scans. This means they can remove as much of the tumor as possible, and sometimes eliminate additional surgeries.
  • Expert neuropathological review, using advanced molecular diagnostic testing, to identify your child’s exact type of tumor. This information helps predict which treatments are more likely to work.
  • Access to unique Phase I clinical trials, from our own investigators, the Children’s Oncology Group and the Pediatric Oncology Experimental Therapeutics Investigators Consortium. Studies offer treatment options beyond standard therapy.
  • Ongoing care from pediatric neurologists familiar with the early symptoms and side effects of brain tumors and their treatments.

Access to one of the nation’s few dedicated pediatric brain tumor survivorship programs. This weekly clinic offers ongoing care to manage late effects caused by your child’s tumor or the treatment they received.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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