What is ependymoma?

Ependymoma is a rare type of tumor of the brain or spinal cord. It can happen in both children and adults. In children, these tumors tend to be in the brain. In adults, they are often in the spinal cord.

Your brain and your spinal cord make up your central nervous system (CNS). Ependymoma is a primary CNS tumor. This means that it starts in the brain or spinal cord rather than starting somewhere else in the body and spreading to the brain.

Ependymoma is a kind of glioma. This is a tumor that develops from support cells (glial cells) of the brain. These tumors arise from abnormal growth of a certain type of glial cells, the ependymal cells. These cells line the ventricles of the brain. The ventricles are the chambers that contain the cerebrospinal fluid (CSF) that bathes and cushions the brain and spinal cord. Ependymomas may spread within the CSF to other places in the brain or spinal cord. These tumors don’t typically spread beyond the CNS.

Most ependymomas are relatively slow-growing tumors. There are 4 types:

• Subependymoma is a usually slow-growing, benign tumor of the ventricles in adults
• Myxopapillary ependymoma is a slow-growing spinal cord tumor usually in young adults
• Classic ependymoma is a somewhat faster-growing tumor in both children and adults
• Anaplastic ependymoma is a faster-growing tumor often near the base of the brain

A tumor in the brain may cause problems by pressing against part of the brain and causing symptoms.

What causes ependymoma?

Researchers do not yet know what causes these tumors. Healthcare providers also do not have a clear idea about the risk factors for these tumors. Certain viruses may play a role in causing these tumors.

What are the symptoms of ependymoma?

The symptoms depend on where the tumor is. For example, a tumor near the base of the brain may block the normal flow of CSF. This can lead to increased intracranial pressure (ICP). This may cause you to have a headache, nausea, vomiting, and dizziness. Your symptoms may be sudden or they may start slowly and gradually worsen. People with spinal cord tumors may have localized pain for months or even years before other symptoms develop. In general, signs and symptoms of ependymoma can include:

• Headache
• Nausea and vomiting
• Pain that wakes you up
• Dizziness and balance problems
• Eye problems, such as double or blurry vision
• Weakness or numbness in an arm or leg
• Difficulty walking
• Bowel or bladder problems

Your symptoms may vary.

How is ependymoma diagnosed?

Ependymoma can be difficult to diagnose because it is a rare tumor in adults. It may be hard to tell the difference from other types of tumors. Your primary healthcare provider may refer you to a neurologist. This is a healthcare provider who specializes in diagnosing and treating diseases of the central nervous system. You may see a neuro-oncologist. This is a healthcare provider who specializes in cancers of the brain and spinal cord. You may be referred to a neurosurgeon. This is a surgeon who performs brain or spinal cord surgery.

 The process starts with a medical history and a physical exam. Your healthcare provider will ask about your symptoms and past medical conditions. He or she may also ask about your family’s medical history. The physical exam may include a neurologic exam. During a neurologic exam, your healthcare provider may ask you to do things like walk, touch your finger to your nose, or hold your hands out. He or she may tell you to follow a light with your eyes. Tests may be done, such as:              

• Magnetic resonance imaging (MRI). This test uses large magnets and a computer to create images of the body. MRI scans of your brain and spinal cord may be done to get more information. Contrast dye may be used to help show more detail in the images.              
• Surgery. The tumor may be removed so that it can be examined. It will be assessed for the type and grade.
• Lumbar puncture. A small amount of CSF is taken from the space around the spine in the lower back. The fluid is checked for tumor cells.

Your healthcare provider will put together a pathology report. This report tells the size, location, type, grade, and other specific information about your tumor.

How is ependymoma treated?

You and your medical team will decide on the best treatment plan for you. These team members may include:

• Neurologist or neuro-oncologist
• Neurosurgeon who is a specialist in the removal of ependymomas
• Radiation oncologist (a healthcare provider who specializes in treating tumors with radiation)
• Nurse
• Nurse practitioner
• Psychologist
• Social worker

Treatment depends on the type and location of your tumor. Your healthcare team will decide the best treatment plan for you. The main treatment in adults is surgery to remove as much of the tumor as possible. Your chances of the best outcome are highest if your healthcare provider is able to fully remove your tumor. A healthcare provider will examine the tumor to find out your tumor type and grade.

Radiation therapy often follows surgery. In some cases, you may need chemotherapy after surgery. You may also have one or more of the following treatments:

• Conformal radiotherapy or intensity-modulated radiotherapy
• Proton beam radiotherapy
• Stereotactic radiosurgery, if the tumor is small enough and has not spread
• Chemotherapy medicine that is given by mouth, IV, or injected into the spinal fluid (intrathecal), for tumors that have spread

You may also need a tube inserted to drain excess CSF and lower your ICP. You will likely need to have several follow-up MRI scans to monitor your situation.

Most people with ependymomas have a good outcome. In some cases, ependymoma comes back after treatment. To check for this, you will likely need to have follow-up MRI scans in the weeks after surgery, and then a few times a year after that. Talk to your healthcare provider about the risk of treatment risks and side effects, your prognosis, and the chances of your tumor returning.

Key points about ependymoma

• Ependymoma is a rare type of primary CNS tumor. It can occur in both children and adults.
• These tumors develop from ependymal cells. These cells line the CSF-filled ventricles of the brain.
• Most ependymomas are slow-growing tumors.
• Researchers do not yet know what causes ependymomas.
• Symptoms depend on where the tumor is. Your symptoms may be sudden, or they may start slowly and get worse over time. Common symptoms include headache, nausea, vomiting, eye problems, dizziness, numbness, bowel or bladder problems, and pain that wakes you up.
• The tumor may be seen on an MRI scan.
• You may need surgery to remove as much of the tumor as possible.
• You may need radiation therapy after surgery. You may also need chemotherapy.
• Your medical team will watch you for to see if the tumor comes back.
• Most people treated for ependymoma have a good outcome.

Next steps

• Know the reason for your visit and what you want to happen.
• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your provider tells you.
• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
• Ask if your condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if you do not take the medicine or have the test or procedure.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your provider if you have questions.