Side view of man’s head showing normal brain.Ependymoma is a rare type of tumor of the brain or spinal cord. It can occur in both children and adults. In adults, it is often in the spinal cord. The tumor may not be cancer (benign), or it may be cancer (malignant). A malignant ependymoma can spread, a benign one cannot.

How does an ependymoma grow?

Your brain and your spinal cord make up your central nervous system (CNS). Ependymoma is a primary CNS tumor. This means it starts in the brain or spinal cord instead of starting elsewhere in the body and spreading to the brain. Any tumor in the brain may cause problems by pressing against part of the brain and causing symptoms.

Ependymoma is a kind of glioma. This is a tumor that starts in support cells (glial cells) of the brain. This tumor is an abnormal growth of a type of glial cell called ependymal cells. These cells line chambers of the brain called ventricles. The ventricles contain the cerebrospinal fluid (CSF). This is the fluid that surrounds and protects the brain and spinal cord. The tumor cells may spread in the CSF to other places in the brain or spinal cord. These tumors don’t often spread beyond the central nervous system.

Ependymomas are grouped by size and shape of the cells, how fast they grow, and where they are, as well as tumor size, consistency, and spread.

There are 4 types of ependymoma tumors:

  • Subependymoma (Grade I). This is a benign tumor of the ventricles. It usually grows slowly.

  • Myxopapillary ependymoma (Grade I). This is a spinal cord tumor. It usually grows slowly.

  • Classic ependymoma (Grade II). This type is most common. It grows a bit faster.

  • Anaplastic ependymoma (Grade III). This is a tumor often near the base of the brain. It also grows faster.

What causes an ependymoma?

Researchers do not yet know what causes these tumors. Certain viruses may be linked to them. The risk factors for these kinds of tumors aren’t known. There's no known way to prevent them.

What are the symptoms of an ependymoma?

Symptoms can vary from person to person. The symptoms also 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 pressure in your skull. This may cause headaches, nausea, vomiting, and dizziness. Your symptoms may be sudden or they may start slowly and get worse over time. In general, signs and symptoms of ependymoma can include:

  • Headache

  • Nausea and vomiting

  • Top view cross section of brain showing ventricles.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

  • Trouble walking

  • Bowel or bladder problems

  • Back pain that spreads to the arms or legs

How is an ependymoma diagnosed?

Ependymoma can be hard to diagnose. This is because it is a rare tumor in adults. It may be hard to tell the difference from other types of tumors. Your healthcare provider may refer you to a neurologist. This is a doctor who specializes in diseases of the central nervous system. You may see a neuro-oncologist. This is a doctor who specializes in cancers of the brain and spinal cord. Or you may be referred to a neurosurgeon. This is a surgeon who does 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 medical history. 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 doctor may ask you to do things such as 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. You may also have tests such as:

  • 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.          

  • 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.

How is an ependymoma treated? 

Your healthcare team will work with you to create a treatment plan. Your team may include:

  • Neurologist or neuro-oncologist

  • Neurosurgeon

  • Radiation oncologist (a doctor who specializes in treating tumors with radiation)

  • Nurse

  • Nurse practitioner

  • Psychologist

  • Social worker

Treatment depends on the type and location of your tumor. The main treatment is surgery. This is done to remove as much of the tumor as possible. A doctor will examine the tumor to find out the type, the size of the tumor, and more information.

You may need radiation therapy or chemotherapy after surgery. Radiation therapy is done by sending radiation to the site of the cancer. Chemotherapy is done with medicines. They may be given by mouth, IV, or injected into the spinal fluid (intrathecal).

You may also need a tube inserted to drain excess CSF and lower the pressure inside your skull.

Follow-up care

In some cases, an ependymoma comes back after treatment. You will likely need to have follow-up MRI scans in the weeks after surgery, and then a few times a year after that.