This information is about a specific type of tumour called an ependymoma. It should ideally be read with our general information about brain or spinal cord tumours.

The central nervous system (CNS) is made up of the brain and the spinal cord. Cells within the CNS normally grow in an orderly and controlled way. If for some reason this order is disrupted, the cells continue to divide and form a lump or tumour.

A tumour may be either benign or malignant. Although a benign tumour can continue to grow, 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.

Tumours affecting the CNS are fairly rare. About 4500 people are diagnosed with tumours of the CNS each year in the UK. Many of these tumours are malignant.

Within the brain and spinal cord, there are nerve cells and also 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, and in children are more common in the cerebellum (see diagram).

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.

People of all ages can develop ependymomas, including children. Tumours at the base of the brain, in an area called the posterior fossa, are more common in children. Ependymomas of the spine account for over half of all adult spinal tumours.

Grading refers to the appearance of the tumour cells under the 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.

As with most brain tumours, the cause of ependymomas is unknown. Research is being carried out into possible causes.

Ependymomas are often slow growing tumours and any signs and symptoms usually develop slowly over many months.

The main symptoms occur due to increased pressure within the skull (known as raised intracranial pressure). This may be caused by a blockage in the ventricles (fluid-filled spaces in the brain) that leads to a build-up of cerebral spinal fluid (CSF). CSF is the fluid that surrounds and protects the brain and spinal cord. The increased pressure may also be caused by swelling due to the tumour itself.

Raised intracranial pressure can cause headaches, sickness (vomiting) and sight changes.

Other specific symptoms of ependymomas include swelling of the nerve at the back of the eye (papilloedema), rapid, jerky eye movements (nystagmus), neck pain and irritability.

Fits (seizures) and changes in behaviour and personality may be general signs of a brain tumour.

Ependymomas can grow in different parts of the brain, and symptoms may relate to the area of the brain that is affected:

• A tumour of 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.

So that your doctors can plan your treatment, they need to find out as much as possible about the type, position and size of the tumour, by doing a number of tests and investigations.

Neurological examination (nerve tests) Usually, you will have a neurological examination to assess any effect of the tumour on your nervous system. A CT scan or an MRI scan will be done to find the exact position and size of the tumour.

CT (computerised tomography) scan A CT scan takes a series of x-rays which build up a three-dimensional picture of the inside of the body. The scan is painless and takes from 10–30 minutes. CT scans use a small amount of radiation, which will be very unlikely to harm you and will not harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.

Most people who have a CT scan are given a drink or injection to allow particular areas to be seen more clearly. This may make you feel hot all over. Before having the injection or drink, it is important to tell the person doing this test if you are allergic to iodine or have asthma.

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. During the scan you will be asked to lie very still on a couch inside a long tube for about 30 minutes. It is painless, but can be uncomfortable, and some people feel a bit claustrophobic during the scan. It is also noisy, but you will be given earplugs or headphones.

Some people are given an injection of dye into a vein in the arm, but this usually does not cause any discomfort.

Biopsy To give an exact diagnosis, a sample of cells from the tumour is sometimes taken, which is then looked at under a microscope. The biopsy involves an operation. Your doctor will discuss with you whether this is necessary in your case, and exactly what the operation involves.

Lumbar puncture Sometimes it is necessary to carry out a test known as a lumbar puncture. The skin on your back is numbed with local anaesthetic, and a needle is passed through the skin and into your spine, so that a small amount of CSF can be withdrawn for tests. The spinal fluid (CSF) can then be examined to see if there are any tumour cells present. MRI scans can also show this.

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 your treatment.

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 cancer (an oncologist), a specialist nurse and possibly other health professionals, such as a physiotherapist or a dietitian.

If the pressure in the skull is raised, it is 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 due to a build-up of CSF, a tube (shunt) may have to be inserted to drain off the excess fluid.

Consent

Before you have any treatment, your doctor will give you full information about what the treatment involves and explain the aims of the treatment to you. They will usually 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. You don’t have to give a reason for not wanting to have treatment, but it can be helpful to let the staff know your concerns so that they can give you the best advice.

Surgery

Where possible, surgery is the preferred 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, cannot be reached by surgery, or the risk of damage to the surrounding brain cells may be too high. If surgery is not possible, your doctor will discuss other types of treatment with you.

Radiotherapy

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.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs that destroy cancer cells. It may be given alone or together with surgery and/or radiotherapy to treat ependymoma.

Chemotherapy may be given to children under the age of three instead of radiotherapy, as there is a lower risk of long-term side effects with chemotherapy in young children.

You may find the idea of a tumour affecting your brain extremely frightening. The brain controls the body, and not being in control is something that can be very worrying. You may experience many different emotions, including anxiety 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 support service nurses. Close friends and family members can also offer support.

Driving

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 one to two years, depending on the grade of your tumour. However, if the tumour comes back this period will be extended.

You may not be allowed to drive some vehicles, such as a LGV (large goods vehicle) or a PCV (passenger carrying vehicle).

The hospital will not contact the DVLA, but it is your responsibility to do so and your doctor will advise you how to do this.

This section has been compiled using information from a number of reliable sources, including:

• Oxford Textbook of Oncology (2nd edition). Souhami et al. Oxford University Press, 2002.
• The Textbook of Uncommon Cancers (3rd edition). Raghavan et al. Wiley, 2006.
• Cancer in the Nervous System (2nd edition). Levin. Oxford University Press, 2002.

For further references, please see the general bibliography.