What is ependymoma?

Ependymoma is a very rare type of tumor that starts in the brain or spinal cord. It can happen in both children and adults.

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 means it starts in support cells (glial cells) of the brain. Ependymomas start when certain types of glial cell called ependymal cells start to grow out of control. 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 can spread when the CSF carries ependymoma cells to other places in the brain or spinal cord. These tumors rarely spread beyond the CNS to other parts of the body.

There are different types of ependymomas. Most tend to be slow-growing tumors. They're given a grade based on how different the tumor cells are compared to normal ependymal cells. A scale of 1 to 3 and Roman numerals are used: I (1), II (2), or III (3). Grade I is the slowest-growing ependymoma, Grade III is fast-growing. These are the major types:

• Subependymoma (grade I). This is a slow-growing tumor near the ventricles. It's more common in adults than children. Many times, it doesn't cause symptoms.

• Myxopapillary ependymoma (grade I). This is a slow-growing, lower spinal cord tumor most common in male adults.

• Classic ependymoma (grade II). This is a somewhat faster-growing tumor in both children and adults. There are many subtypes.

• Anaplastic ependymoma (grade III). This is a fast-growing cancer tumor often in the base of the brain and rarely in the spinal cord. It tends to spread into nearby parts of the brain and spread to other parts of the brain through the CSF. These tumors tend to come back (recur) after treatment.

Some grade I ependymomas are not called cancer because they grow slowly and don't spread. Still, any tumor in the brain, no matter how small, may cause problems by pressing against parts of the brain and causing symptoms. An ependymoma that causes symptoms will be treated, even if it's not cancer.

What causes ependymoma?

Researchers don't yet know what causes these tumors. Healthcare providers also don't have a clear idea about the risk factors for these tumors.

Studies have shown that people with a genetic disorder called neurofibromatosis type 2 (NF2) have a higher risk of developing an ependymoma.

What are the symptoms of ependymoma?

The symptoms depend on where the tumor is. For instance, a tumor near the base of the brain may block the normal flow of CSF. This can lead to increased intracranial pressure (ICP, which is the pressure inside your head), which can cause headaches, nausea, vomiting, and dizziness. Symptoms may be sudden or they may start slowly and get worse over time. People with spinal cord tumors may have pain where the tumor is for months or even years before other symptoms develop.

Common 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

• Trouble with balance or walking

• Bowel or bladder problems

• Seizures

How is ependymoma diagnosed?

Ependymoma can be hard to diagnose because it's 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 provider 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.

You'll be asked about your symptoms and health history. The physical exam that includes a nervous system exam will be done. During a nervous system exam, you'll be asked to do things like walk, touch your finger to your nose, or hold your hands out. You may be asked to follow a light with your eyes.

If a tumor is suspected, other tests may be done, such as:              

• MRI. This test uses large magnets and a computer to create images of the inside of your body. MRI scans of your brain and spinal cord can be used to look for a tumor or learn more about it, such as where it is, how big it is, and if it has spread. 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 then be tested to find out the type and grade.

• Spinal tap (lumbar puncture). A small amount of CSF is taken from the space around your spine by putting a thin needle between the bones of your lower back. CSF is pulled into a syringe attached to the needle and checked for tumor cells.

These tests are used to find out the size, location, type, grade, and other details about the tumor.

How is ependymoma treated?

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

• Neurologist or neuro-oncologist

• Neurosurgeon who has experience removing ependymomas

• Radiation oncologist. This is a healthcare provider who specializes in treating tumors with radiation.

• Nurse

• Nurse practitioner

• Psychologist

• Social worker

Treatment depends on the type of tumor and where it is. Your healthcare team will help you 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 the tumor can be fully removed.

Radiation therapy might be used after surgery. If the tumor has spread, which is rare, chemotherapy may be needed after surgery. It can be given as a pill or put right into a vein by IV (intravenous) line into your blood. A needle can be used to put chemo into your CSF (spinal fluid), too. (This is called intrathecal chemo.)

Prognosis means the likely outcome of the cancer. Most people with an ependymoma respond well to treatment and have a good outcome. Sometimes higher grade ependymomas come back after treatment. To check for this, you'll likely need to have follow-up MRI scans in the weeks after surgery, and then a few times a year after that.

Talk with your healthcare provider about 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 don't know what causes ependymomas.

• You may need surgery to remove as much of the tumor as possible. Radiation and chemotherapy are sometimes used after surgery.

• Most people treated for ependymoma have a good outcome.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• 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 don't 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 healthcare provider if you have questions, especially after office hours or on weekends.