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My Child Has:
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Pleomorphic Xanthoastrocytoma
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A pleomorphic xanthoastrocytomas 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. These cells are named for their star-like appearance.
Pleomorphic xanthoastrocytomas generally arise superficially from the cerebral hemispheres (upper most 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 below, 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.
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Medical research has not identified a genetic predisposition to the development of pleomorphic xanthoastroctyomas. Children with these tumors appear to develop them spontaneously, meaning there is no identifiable cause.
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Due to the relatively slow growth rate of pleomorphic xanthoastrocytomas, children with these tumors tend to present to the doctor with symptoms that have been occurring for many months. Some children however, will have a more sudden onset of symptoms. The most common symptom at diagnosis is seizure activity.
The symptoms of a brain tumor may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
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Diagnostic procedures for a pleomorphic xanthoastrocytoma may include:
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- physical examination
- computerized tomography scan (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays. For pleomorphic xanthoastrocytoma, a CT scan of the brain is usually done.
- magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. For pleomorphic xanthoastrocytoma, MRIs of the brain and spine are usually done.
- electroencephalogram (EEG) - for children experiencing seizures, this procedure records the brain's continuous, electrical activity by means of electrodes attached to the scalp, and helps identify and localize seizure activity to a particular section of the brain
- biopsy - a tissue sample from the tumor taken through a needle during a simple surgical procedure performed by a surgeon to confirm the diagnosis
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Specific treatment for a pleomorphic xanthoastrocytoma will be determined by your child's physician based on:
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Specific treatment for a pleomorphic xanthoastrocytoma will be determined by your child's physician based on:
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- your child's age, overall health, and medical history
- type, location, and size of the tumor
- extent of the disease
- your child's tolerance for specific medications, procedures, or therapies
- how your child's doctors expects the disease to progress
- your opinion or preference
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Treatment may include (alone or in combination):
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- surgery - the primary treatment is maximum surgical removal of the tumor. Tumors that are completely removed may not require further therapy other than serial MRIs to monitor for tumor re-growth. For tumors that are incompletely removed and then increase in size or for tumors that recur, a reattempt at surgical removal will be recommended.
- radiation therapy - may also be recommended although data suggests that radiation therapy does not influence long-term outcome. Radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors.
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Children with pleomorphic xanthoastrocytomas may have side effects related to the tumor itself and its treatment. If there are symptoms at the time of diagnosis related to increased pressure within the brain, surgical removal of the tumor will likely reduce these symptoms. Dexamethasone, an oral steroid, is often used, especially in the post-operative period, to assist in controlling systems of increased pressure within the brain, as well as any additional tissue swelling that may occur post-operatively.
Children with seizures are treated with anti-seizure medications to control or, in some cases, eliminate these symptoms.
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Pleomorphic xanthoastrocytomas are associated with a high rate of cure. Data suggest a long-term survival rate of approximately 90 percent. Greater degree of surgical removal appears to improve long-term survival.
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The recommended treatment for progressive/recurrent pleomorphic xanthoastrocytomas is reattempt at surgical removal. Dana-Farber Cancer Institute is one of nine institutes in the nation belonging to the Pediatric Brain Tumor Consortium. The consortium is dedicated to the development of new and innovative treatments for children with progressive/recurrent brain tumors not responsive to standard therapies. Children with pleomorphic xanthoastrocytomas would be eligible for a number of experimental therapies available through the consortium.
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Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat pleomorphic xanthoastrocytoma. For more information on current research, see the Brain Tumor Program.
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