Ependymoma

What is an ependymoma?

An ependymoma is a mass of irregular cells (tumor) in your brain or spinal cord. It’s a type of primary central nervous system tumor. Ependymomas start in your brain or spinal cord instead of starting elsewhere and spreading to your brain or spinal cord. Ependymomas tend to grow slowly and usually don’t spread to other parts of your body.

An ependymoma is a type of glioma, meaning it starts in glial cells. Glial cells help support the neurons in your brain and spinal cord.

What are the types of ependymomas?

Healthcare providers rate ependymomas on a 1 to 3 grading scale, with grade 1 tumors growing the slowest and grade 3 tumors growing the fastest. There are several types of ependymomas, including:

• Subependymoma (grade 1) grows near the chambers (ventricles) that contain cerebrospinal fluid. It’s more common in adults than children.
• Myxopapillary ependymoma (grade 1) grows in your lower spinal cord and is most common in adult men.
• Classic ependymoma (grade 2) is the most common ependymoma and occurs in children and adults.
• Anaplastic ependymoma (grade 3) often grows near the base of your brain and spreads to other parts of your brain. These tumors tend to return (recur) after treatment.

Who is most likely to get an ependymoma?

Ependymomas are more common in children than adults. They’re the sixth most common type of brain tumor in children.

How common are ependymomas?

Experts estimate that nearly 22 in 100,000 people have primary central nervous system tumors. Of all primary central nervous system tumor diagnoses, ependymomas make up:

• Less than 2% of adult diagnoses.
• Around 4% of diagnoses in children ages 15 to 19.
• Almost 6% of diagnoses in children from birth to 14.

What are the symptoms of ependymoma?

Ependymoma symptoms vary depending on the tumor location and size and your age. In babies, an ependymoma may cause:

• Irregularly large head.
• Irritability.
• Sleeplessness.
• Vomiting.

In older children and adults, ependymomas may cause:

• Back or neck pain.
• Balance problems.
• Blurry vision.
• Difficulty urinating.
• Dizziness.
• Headaches.
• Muscle weakness.
• Numbness in arms or legs.
• Nausea or vomiting.
• Seizures.

What causes an ependymoma?

Experts don’t know what causes ependymoma. In general, cancer develops when specific genes change (mutate). Experts don’t know what causes those gene mutations. Researchers have found that people with neurofibromatosis type 2 (NF2) are more likely to develop an ependymoma.

How is an ependymoma diagnosed?

Diagnosing an ependymoma may require multiple specialists, such as a:

• Neurologist, a doctor specializing in the central nervous system.
• Neuro-oncologist, a doctor specializing in central nervous system tumors.
• Neurosurgeon, a doctor specializing in brain and spinal cord surgery.

You may have tests to learn more about the tumor’s size, location and grade, such as:

• MRIs: Specialists use large magnets and computers to view your brain or spinal cord comprehensively.
• Spinal tap (lumbar puncture): Your healthcare provider removes a small sample of cerebrospinal fluid to test it for tumor cells.
• Surgery: A neurosurgeon may remove the tumor and test it to determine its type and grade.

How is an ependymoma treated?

Your healthcare providers may treat an ependymoma with:

• Surgery: Surgery is the most common ependymoma treatment. Your neurosurgeon removes as much of the tumor as possible while keeping healthy tissue intact. Surgery is often the only treatment you need for an ependymoma.
• Radiation therapy: Your radiation oncologist uses strong energy beams to shrink or destroy tumor cells. Many people have radiation therapy before or after surgery.
• Chemotherapy: You take medicines that destroy fast-growing cells, including tumor cells. You may need chemotherapy if the tumor has spread to other areas of your body, which is rare with ependymomas.
• Immunotherapy: You take drugs that stimulate your immune system to fight cancer more effectively. Immunotherapy is a rare treatment for an ependymoma, but you may need it if the tumor has spread to other parts of your body.
• Targeted therapy: This treatment uses drugs or other substances that target specific cells to attack cancer cells or keep them from growing. It’s rarely used as a treatment for ependymoma.

How can I reduce my risk of developing an ependymoma?

Because experts don’t know what causes ependymomas, there’s no way to prevent them. See your healthcare provider right away if you develop any symptoms that could point to an ependymoma. Treating a tumor is often easier when it’s in the early stages of development.

What is the prognosis for ependymoma?

The five-year survival rate for ependymoma is nearly 84%. However, multiple factors affect prognosis, including the tumor grade, location and your health and age.

What questions should I ask my doctor?

If you or your child has an ependymoma, you may want to ask your healthcare provider:

• What tests are needed to diagnose ependymoma?
• Does my child or do I need tests to check for neurofibromatosis?
• What are the treatments for ependymoma?
• What are the chances that an ependymoma will return after treatment?

What is the 10-year survival rate for ependymomas?

Ependymoma survival rates are more favorable in adults than children. The 10-year survival rate is approximately 70% to 89% in adults. In children, it’s about 64%.

A note from Cleveland Clinic

An ependymoma is a tumor that starts in your brain or spinal cord. It can develop in children and adults, although it’s more common in children. There are many types and grades of ependymomas. The tumor location and grade can affect what symptoms you experience. People with ependymoma may experience balance problems, headaches, muscle weakness or numbness. Treatment usually involves surgery to remove the tumor.