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Ependymomas
What Is an Ependymoma?
An ependymoma (ep-en-deh-MO-muh) is a group of abnormal cells (a tumor) that forms in the brain or spinal cord. Most ependymomas are treated with surgery and radiation therapy. Some children may also have chemotherapy (chemo).
What Happens With an Ependymoma?
Cells called ependymal (ep-EN-deh-mul) cells line the spinal cord and ventricles (spaces) in the brain. Sometimes ependymal cells become abnormal and group together. When this happens, it forms an ependymoma.
Most ependymomas in children are benign (they grow slowly and aren't likely to spread), but they can be cancerous (they grow quickly and are likely to spread). Ependymomas that spread can move to other parts of the brain and spinal cord through cerebral spinal fluid (the liquid that surrounds the brain and spinal cord). The tumors don’t usually move to other parts of the body.
What Causes Ependymomas?
Doctors aren’t sure what causes ependymomas. People with a genetic condition called neurofibromatosis type 2 (or NF2, which causes benign tumors to grow in and under the skin and other problems) are more likely to have them.
What Are the Types of Ependymomas?
There are many different kinds of ependymomas. In kids, the tumors usually happen in the brain but can also grow in the spinal cord. Doctors group ependymomas by where the tumor is, how aggressive it is (how fast it grows), and changes in the DNA of the tumor’s cells.
What Are Signs & Symptoms of Ependymomas?
The signs and symptoms of an ependymoma depend on where it is and how old the child is. An ependymoma in the brain can lead to:
- nausea and/or vomiting
- headaches or blurry vision
- confusion
- neck pain
- seizures
- balance problems
- crankiness
An ependymoma in the spinal cord can lead to:
- back pain
- leg weakness
- problems urinating (peeing) or pooping
How Are Ependymomas Diagnosed?
To diagnose an ependymoma, doctors will ask about symptoms and do an exam. They’ll also order imaging tests like an MRI. These tests let them look inside the brain or spinal cord to see if any areas look different.
Sometimes the ependymoma can be diagnosed from the exam and imaging tests. Other times, it may not be clear what kind of tumor it is, so a biopsy may be needed. With this test, doctors take a piece of the tumor and look at it under a microscope. They may use a needle or do the biopsy during surgery to remove the tumor.
Ependymoma cells can spread throughout the brain and spine, so doctors will do more tests, including a spinal tap. This lets them get a small sample of cerebral spinal fluid to check.
When the doctors have the information they need, they can create a treatment plan.
How Are Ependymomas Treated?
Treatment for an ependymoma usually starts with surgery to remove the tumor as well as radiation therapy. Some children may also have chemo.
After finishing treatment, kids will need regular follow-up so doctors can see how they’re doing. Because an ependymoma can come back, kids will need an MRI several times a year for the first few years, then less often. This helps doctors find any problems early so they can work with families to make a treatment plan.
Doctors are looking for new ways to treat ependymomas. Some kids might be able to join a clinical trial. This is a study researchers do to see whether a new treatment works well and is safe. To find out if it or a different treatment is a good choice for your child, talk with their doctor.
Who Is on the Care Team?
A team of specialists works together to care for a child with an ependymoma. They may include:
- a pediatric neuro-oncologist (a doctor who treats cancers of the brain or spine)
- a pediatric neurologist (a doctor who treats nervous system problems)
- a pediatric neurosurgeon (a surgeon who operates on the brain or spine)
- a pediatric radiation oncologist (a specialist who gives radiation therapy)
- pediatric rehabilitation medicine specialists, including speech therapists, physical therapists, and occupational therapists
- pediatric psychologists and social workers
What Else Should I Know?
If your child has an ependymoma, it can help to find support from members of the care team, friends and family, and other parents who’ve gone through the same thing.
When talking with your child, it’s best to be honest. But give information that fits their age and emotional maturity. Make sure kids know that the tumor isn’t because of anything they did. If you need help answering questions or don’t know what to say, a member of the care team can help.
The future for kids with brain tumors is better than ever. You can find more information and support online at:
Note: All information is for educational purposes only. For specific medical advice,
diagnoses, and treatment, consult your doctor.
© 1995- The Nemours Foundation. KidsHealth® is a registered trademark of The Nemours Foundation. All rights reserved.
Images sourced by The Nemours Foundation and Getty Images.
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