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 brain and spinal cord make up the central nervous system (CNS). The brain controls different functions of the body, how we think, feel, learn and move. The spinal cord is made up of nerves that run down the middle of the back (spine). Messages between the brain and other parts of the body travel through the spinal cord.
A tumour can be benign (not cancer) or malignant (cancerous). A benign brain tumour may cause problems as it grows because it can press on surrounding tissue. But it can’t spread to other parts of the brain.
A malignant tumour can cause problems by growing into nearby tissues and may spread to other parts of the CNS. Slow-growing ependymomas may sometimes be called benign tumours.
Ependymomas are rare. They make up less than 4% of all CNS tumours in adults. They are a type of tumour called a glioma, which starts in the glial cells. Glial cells support and protect nerve cells in the brain (neurons). Ependymomas develop from a sub-type of glial cells called ependymal cells. These cells line the fluid-filled spaces in the brain (ventricles) and the centre of the spinal cord.
Ependymomas are often slow-growing tumours. They can develop in adults (usually adults under 45 years old) and also in children. This information is about ependymomas in adults. We have more information about brain tumours in children.
Ependymomas can develop in any part of the brain or spine. Occasionally, ependymomas that start in the brain can spread through the cerebrospinal fluid (CSF) to the spinal cord. The CSF fluid surrounds and protects the brain and spinal cord.
Causes of an ependymoma
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As with most CNS tumours, it’s not known what causes ependymomas but research is going on to find out more. Previous radiation to the head or a genetic (hereditary) condition called type II neurofibromatosis may be risk factors.
Symptoms of an ependymoma
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Because ependymomas are often slow-growing tumours, the symptoms usually develop slowly over many months. The symptoms will depend on whether it is in the spinal cord or the brain.
If the tumour is in the spine, pain in the neck or back is usually the first symptom. Other symptoms are numbness or weakness in the arms or in the legs, or problems with bladder control. We have more information about spinal tumours.
If the ependymoma is in the brain, symptoms will depend on the part of the brain that is affected by the tumour.
The first symptoms of ependymoma in the brain may be due to increased pressure in the brain (called raised intracranial pressure). This can happen because there is swelling around the tumour, or a build-up of cerebrospinal fluid (CSF) that surrounds and protects the brain and spinal cord. The symptoms of raised intracranial pressure are headaches, sickness (vomiting), problems with balance and with sight, and being confused.
Some other possible symptoms are:
changes in mood and personality
weakness in an arm or leg
problems with coordination and balance
Your doctors will need to find out as much as possible about the type, position and size of the tumour, so they can plan the best treatment for you. You will have a number of different tests.
The doctor will examine you and do checks on your nervous system. This includes checking your reflexes and the power and feeling in your arms and legs. They also shine a light at the back of your eye to check if the nerve (optic nerve) is swollen, which can be a sign of raised pressure in the brain. Your doctor will ask some questions to check your reasoning and memory. You will also have blood tests to check your general health and to see how well your kidneys and liver are working.
You will have a CT scan or MRI scan to find out the exact position and size of the tumour.
MRI (magnetic resonance imaging) scan
This test uses magnetism to build up a detailed picture of the brain and spinal cord.
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.
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 and usually only takes minutes. CT scans use small amounts of radiation, which are 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.
Your doctor will explain if you need to have a biopsy (removing a small piece of the tumour). The biopsy and surgery to remove the tumour are sometimes done at the same time.
If you have a biopsy, it will usually be done under a general anaesthetic. The neuro (brain) surgeon makes a small hole in the skull and passes a fine needle through into the tumour. They remove a small sample of tissue which is examined to find out the type of cells the tumour is made up of.
A lumbar puncture is sometimes carried out after ependymoma is diagnosed to look for tumour cells in the cerebrospinal fluid (CSF).
Your doctor uses a local anaesthetic to numb the lower part of your back and then passes a needle gently into the spine. They then take a small sample of CSF to be checked for tumour cells.
A lumbar puncture is usually done as an outpatient and it only takes a few minutes.
MRI scans can also show if the tumour is in the spinal cord.
Grading of an ependymoma
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Grading is about how the tumour cells look when they’re examined by a doctor (pathologist) under a microscope. The grade can give an idea of how quickly the tumour may grow. There are three grades of ependymoma.
The grades are:
grade 1 (called myxopapillary ependymomas and subependymomas) usually grow slowly and are unlikely to come back if they are completely removed
grade 2 (low-grade ependymoma) may grow more quickly than grade 1
grade 3 (malignant or anaplastic ependymoma) are likely to grow faster than grades 1 or 2.
Treatment for an ependymoma
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The main treatments for an ependymoma are surgery and radiotherapy. Your treatment will depend on the size and position of the tumour, and your general health.
Your specialist doctor and nurse will explain the aims of your treatment and what it involves. They will talk to you about the benefits and disadvantages of different treatment types. They will also explain the risks and side effects.
You and your doctor can decide on the treatments that are right for you. Make sure you have enough information and time to help you make a decision about your treatment.
Surgery is the main treatment for ependymoma. The aim is to get rid of all or as much of the tumour as possible.
If you have a low grade ependymoma that has been completely removed, you will not usually need any other treatment. If the surgeon cannot remove the tumour completely, they will usually advise you to have radiotherapy to get rid of any remaining cells.
Your surgeon will explain what your operation will involve. They will talk to you about the possible complications and risks.
It can take a while to recover after surgery so it’s important to take good care of yourself. Get plenty of rest, try to eat well and follow the advice given by your surgeon and specialist nurse.
Some people may need extra support to help with their recovery. This may be from a physiotherapist who can help you to improve your balance, walking or strength. Occupational therapists can provide equipment and help you become more independent.
Sometimes an operation is not possible. This may be because the position of the tumour makes it too difficult to reach and surgery would not be safe. Radiotherapy can be used instead.
Radiotherapy treatment uses high energy rays to destroy the tumour cells. Your cancer doctor (oncologist) will tell you how long your treatment will last and the type of radiotherapy you will have. Radiotherapy may be used:
on its own when surgery is not possible
after surgery for ependymomas that could not be completely removed
after surgery and depending on the grade of the tumour, to help reduce the risk of it coming back, even when it has all been removed.
Radiotherapy makes you feel very tired and this can carry on for weeks or longer after it finishes. Try to get plenty of rest. The skin on your scalp or spine may become itchy and red or darker. You will lose the hair on in the area being treated. It usually grows back again after 2-3 months. Your nurse will give you advice about looking after the skin on your scalp and coping with hair loss.
Your cancer doctor and specialist nurse will talk to you about the immediate and possible long-term side effects of radiotherapy. Newer ways of giving radiotherapy aim to give a higher dose of radiotherapy to the tumour without damaging the surrounding normal brain.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. In adults with ependymoma, chemotherapy is not commonly used as part of first treatment. Doctors may suggest having chemotherapy if an ependymoma comes back.
Steroid drugs reduce the swelling that surrounds a tumour. You may be given them before or after surgery and radiotherapy. They improve your symptoms and help you feel better.
Some of the side effects of steroids include: indigestion, weight gain, restlessness, agitation and sleep disturbance. Let your doctor or nurse know if these are causing problems or you notice any other effects. Taking steroids with food can help reduce indigestion.
It’s very important to take steroids exactly as your doctor has prescribed them.
Medicines for seizures
If you have seizures (fits) you will be given drugs called anticonvulsants to help prevent them.
Being diagnosed with a brain tumour may feel frightening. You may have many different emotions, including anxiety, anger, fear, feeling low or depressed. Many people go through these in coping with their illness.
It’s important to get the support you need. You may find it helpful to talk things over with family and close friends and with your doctor or nurse. You can also talk to one of our cancer support specialists. Sometimes people need more help to cope with difficult feelings of anxiety or depression. Your doctor can refer you to a counsellor or psychologist for more support.
You may not be allowed to drive for a period of time depending on the treatment you have had and if you have had any fits (seizures). Although this can be upsetting it’s important to follow the advice you are given. Your doctor will ask you to contact the Drivers and Vehicle Licensing Association (DVLA) if you live in England, Scotland or Wales. If you live in Northern Ireland you will need to contact the Driver and Vehicle Agency (DVA). It is your responsibility to contact the DVLA or DVA. Your doctor or nurse will explain what you need to do.
This information has been compiled using a number of reliable sources, including:
Kaye A, Laws ER. Brain Tumours. 2012. Saunders Elsevier.
Packer RJ, Schiff D. Neuro-oncology. 2012. Wiley-Blackwell.
Shahid Iqbal M, Lewis J. An Overview of the Management of Adult Ependymomas with Emphasis on Relapsed Disease. Clinical Oncology. 2013. 25: 726
Thank you to Mr Andrew Brodbelt, Consultant Neurosurgeon, who reviewed this information.
Thank you to all of the people affected by cancer who reviewed what you're reading and have helped our information to grow.
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