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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 .

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. They do not spread to other parts of the body, such as organs outside the central nervous system.

Causes Of An Ependymoma

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

Signs And Symptoms

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. But fits are not a common symptom of ependymomas.

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.

Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma.

The following tests and procedures may be used: -
  • CT scan (CAT scan) : - A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging) with gadolinium : -A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).

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Tests And Investigations

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.

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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.

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