An ependymoma is a rare type of brain tumour.
This information describes ependymomas, their symptoms, tests and possible treatments. It should ideally be read with our general information about brain and spinal cord tumours.
We hope it answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having treatment.
The central nervous system (CNS) is made up of the brain and spinal cord. Cells in the CNS normally grow in an orderly and controlled way. If for some reason this process gets out of control, the cells continue to divide and form a lump called a tumour.
A tumour may be either benign or malignant. Benign tumours may continue to grow but the cells do not spread from the original site. In a malignant tumour, the cells can invade and destroy surrounding tissue and may spread to other parts of the brain.
Within the brain and spinal cord are nerve cells, and cells that support and protect the nerve cells. The supporting cells are called glial cells. A tumour of these cells is known as a glioma.
Ependymomas are a rare type of glioma. They develop from the ependymal cells, which line the ventricles (fluid-filled spaces in the brain), and from the central canal of the spinal cord. They can be found in any part of the brain or spine. In children, they are more commonly found in the cerebellum (see the diagram below).
Ependymomas may occasionally spread from the brain to the spinal cord in the cerebrospinal fluid (CSF). CSF is the fluid that surrounds and protects the brain and spinal cord.
About 9,000 people are diagnosed with tumours of the CNS each year in the UK. Around 1 in 20 (5%) of these are ependymomas. People of all ages can develop ependymomas, including children. Tumours at the base of the brain, in an area called the posterior fossa, and tumours found in the cerebellum (see diagram below) are more common in children. Ependymomas of the spine are more common in adults.
Causes of an ependymoma
Back to top
The cause of ependymomas is unknown. Research is being carried out into possible causes.
Grading of an ependymoma
Back to top
Grading refers to the appearance of the tumour cells under a microscope.
The grade can give an idea of how quickly the tumour may develop. There are three grades of ependymoma. Grade 1 tumours are usually slow-growing and grade 3 tumours tend to grow more quickly.
The grades are:
grade 1 - myxo-papillary ependymoma and sub-ependymoma
grade 2 - ependymoma (low-grade)
grade 3 - anaplastic (malignant) ependymoma.
Signs and symptoms of an ependymoma
Back to top
Ependymomas are often slow-growing tumours, and any signs and symptoms usually develop slowly over many months. Symptoms include:
Raised intracranial pressure
The main symptoms occur because of increased pressure within the skull, which is known as raised intracranial pressure. This may be caused by a blockage in the ventricles, which then leads to a build-up of cerebral spinal fluid (CSF). The increased pressure may also be caused by swelling due to the tumour itself. Raised intracranial pressure can cause headaches, sickness (vomiting
), neck pain and irritability. It can also cause sight changes and rapid or jerky eye movements (nystagmus).
Seizures (fits) and changes in behaviour and personality may be general signs of a brain tumour.
Changes in brain and nerve function
Ependymomas can grow in different parts of the brain, and symptoms may relate to the area of the brain that is affected:
A tumour in the frontal lobe of the brain may cause gradual changes in mood and personality. There may also be paralysis (the loss of the ability to move) on one side of the body (hemiparesis).
A tumour in the temporal lobe of the brain may cause problems with coordination and speech, and may affect memory.
If the parietal lobe of the brain is affected, writing and other such tasks may be difficult. Hemiparesis may also be present.
An ependymoma in the cerebellum may lead to problems with coordination and balance.
The symptoms of an ependymoma in the spinal cord will depend on which part of the spine is affected. Symptoms include neck or back pain, and sometimes numbness or weakness in the limbs and loss of bladder control.
Tests and investigations for ependymomas
Back to top
Your doctors need to find out as much as possible about the type, position and size of the tumour so they can plan your treatment.
You may have a number of tests and investigations. The doctor will examine you thoroughly and test your reflexes and the power and feeling in your arms and legs.
Your doctor will look into the back of your eyes using an ophthalmoscope to see if the nerve at the back of the eye is swollen. This can be caused by oedema (swelling of the tissues within the brain), which may occur due to an increase in the amount of fluid in the brain.
You will have a CT or MRI scan to find out the exact position and size of the tumour.
CT (computerised tomography) scan
A CT scan takes a series of x-rays that build up a three-dimensional picture of the inside of the body. The scan is painless but takes 10-30 minutes. CT scans use small amounts of radiation, which will be very unlikely to harm you or anyone you come into contact with.
You may be given an injection of a dye, which allows particular areas to be seen more clearly. This may make you feel hot all over for a few minutes. If you’re allergic to iodine or have asthma, you could have a more serious reaction to the injection, so it is important to let your doctor know beforehand.
MRI (magnetic resonance imaging) scan
This test is similar to a CT scan but uses magnetism instead of x-rays to build up a detailed picture of areas of your body.
Before the scan you may be asked to complete and sign a checklist. This is to make sure it’s safe for you to have an MRI scan. Before having the scan, you’ll be asked to remove any metal belongings, including jewellery. Some people are given an injection of a dye into a vein in the arm.
This is called a contrast medium and can help the images from the scan show up more clearly. During the test, you will be asked to lie very still on a couch inside a long cylinder (tube) for about 30 minutes. It is painless but can be slightly uncomfortable, and some people feel a bit claustrophobic during the scan. It’s also noisy, but you’ll be given earplugs or headphones.
A lumbar puncture
is carried out to see if there are any tumour cells present in the cerebrospinal fluid (CSF). The skin on your back is numbed with local anaesthetic, and a hollow needle is inserted between two of the spinal bones and into the spinal canal. A small amount of spinal fluid is withdrawn for tests. MRI scans can also show the presence of any tumour in the spinal cord.
To give an exact diagnosis, a sample of cells (biopsy) is sometimes taken from the tumour, which is then looked at under a microscope. The biopsy involves an operation. Your doctor will discuss whether this is necessary in your case, and exactly what the operation involves. In some situations, the biopsy and surgery to remove the tumour may be done at the same time.
Treatment for an ependymoma
Back to top
The treatment for an ependymoma depends on a number of things including your general health, the size and position of the tumour, and whether it has spread to other parts of the brain or spinal cord. The results of your tests will enable your doctor to decide on the best plan for you.
There are some risks associated with treatment to the brain and your doctor will discuss these with you. Your treatment will usually be planned by a team of specialists known as a multidisciplinary team (MDT).
The team will usually include:
a doctor who operates on the brain (neurosurgeon)
a doctor who specialises in treating illnesses of the brain (neurologist)
a doctor who specialises in treating brain tumours (an oncologist)
a specialist nurse
and possibly other healthcare professionals, such as a physiotherapist or dietitian.
If the pressure in the skull is raised, it’s important to reduce it before any treatment is given for brain tumours. Steroid drugs
may be given to reduce swelling around the tumour. If raised intracranial pressure is because of a build-up of CSF, a tube (shunt) may be inserted to drain off the excess fluid.
Before you have any treatment, your doctor will give you full information about its aims and what it involves. They will ask you to sign a form saying that you give your permission (consent
) for the hospital staff to give you the treatment. No medical treatment can be given without your consent.
Benefits and disadvantages of treatment
Treatment can be given for different reasons and the potential benefits will vary depending upon the individual situation. If you have been offered treatment that aims to cure your cancer, deciding whether to have the treatment may not be difficult. However, if a cure is not possible and the treatment is to control the cancer for a period of time, it may be more difficult to decide whether to go ahead.
If you feel that you can’t make a decision about the treatment when it is first explained to you, you can always ask for more time to decide.
You are free to choose not to have the treatment and the staff can explain what may happen if you do not have it. Although you don’t have to give a reason for not wanting to have treatment, it can be helpful to let the staff know your concerns so that they can give you the best advice.
Surgery, where possible, is the preferred first form of treatment for ependymoma. The aim of surgery is to remove as much of the tumour as possible without damaging the surrounding brain tissue. Depending on the size, position and spread of the tumour, it may not be possible to remove it completely and further treatment may be given as a follow-up to surgery.
Some tumours, particularly those that are high-grade, can’t be reached by surgery or the risk of damage to the surrounding brain cells may be too high. If surgery isn’t possible, your doctor will discuss other types of treatment with you.
Radiotherapy treatment uses high-energy rays to destroy the cancer cells and is often used after surgery to destroy any remaining malignant cells. It is used alone to treat tumours that cannot be reached by surgery. Whenever possible, radiotherapy isn't given to children under the age of three in order to allow further development of the brain and spinal cord, and to reduce the risk of long-term side effects. Instead, a course of chemotherapy is given and, if necessary, radiotherapy when the child is older.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It may be given alone or together with surgery and/or radiotherapy to treat ependymoma.
Medicines for seizures
If you experience seizures, you may be given a medicine called an anticonvulsant to help prevent them.
You may find the idea of a tumour affecting your brain extremely frightening. You may experience many different emotions including anxiety, anger and fear. These are all normal reactions and are part of the process many people go through in trying to come to terms with their condition.
Many people find it helpful to talk things over with their doctor or nurse or with one of our cancer support specialists. Family members and close friends can also offer support.
In some circumstances, you may not be allowed to drive for a period of time. If you have had an epileptic fit, the Drivers and Vehicle Licensing Association (DVLA) will not allow you to drive for a year after your last fit. You can then drive again provided you remain well.
After treatment, you will not be able to drive for at least 1-2 years, depending on the grade of your tumour. However, this period will be extended if the tumour comes back.
You may not be allowed to drive some types of vehicle, such as an LGV (large goods vehicle) or a PCV (passenger carrying vehicle).
It is your responsibility to contact the DVLA and your doctor will advise you how to do this.
This information has been compiled using a number of reliable sources, including:
Bernstein & Berger. Neuro-oncology - the essentials. 2nd edition 2008. Thieme.
Cancer Research UK (CRUK). Cancer Stats. Key facts: Brain and other central nervous system (CNS) tumours. September 2012.
Kieran. Ependymoma. 2012. Up To Date (accessed September 2012).
National Institute for Health and Clinical Excellence (NICE). Improving Outcomes for People with Brain and Other CNS Tumours - The Manual. 2006.
Souhami and Hochhaser. Cancer and its management. 6th edition. 2010. Wiley-Blackwell.
With thanks to: Dr Nick Plowman, Consultant Clinical Oncologist: and the people affected by cancer who reviewed this edition. Reviewing information is just one of the ways you could help when you join our Cancer Voices network.