Learn about ependymoma and its treatment. 

What it is

Ependymoma is rare. It is a type of brain tumour called glioma. They start from ependymal cells. These cells line the fluid filled areas of the brain (ventricles) and the spinal cord. Their job is to repair any damaged nerve tissue. About 2 out of 100 (2%) of brain tumours are ependymomas. 

Most ependymomas are diagnosed in children or young adults and can occur in any part of the brain or spinal cord. In older people they tend to occur in the lower spinal cord.

Ependymomas can be low grade (slow growing) or high grade (fast growing). The appearence of cells under the microscope does not always fit with their behaviour. So the grade may not tell you much about how likely the tumour is to grow or spread. 

Where ependymomas grow

These brain tumours can grow in the brain, or in the spinal cord. They can spread into the fluid that surrounds the brain. So sometimes ependymomas spread into other areas of the lining of the brain and spinal cord.

In children, ependymomas most often occur in the cerebellum. They might develop in the cerebrum, or less commonly in the spinal cord.

Diagnosing ependymoma

Your doctor will examine you and you may have blood tests to check your general health. Other tests include:

• CT or MRI scan 
• biopsy
• lumbar puncture 

Treatment options

Surgery 

Your surgeon will try to remove as much of the tumour as possible. It might be possible to completely remove the ependymoma. 

Radiotherapy

Your might have radiotherapy after surgery, to reduce the risk of the tumour coming back.

You are more likely to have radiotherapy to the whole brain and spinal cord if the fluid around the brain (cerebrospinal fluid) has cancer cells. You might have radiotherapy to the area of the tumour (and not the spinal cord) if there are no cancer cells in the cerebrospinal fluid. 

Children under 3 usually have chemotherapy instead of radiotherapy after surgery. Cancer specialists don't like to treat children under 3 with radiotherapy. Their young age makes them more likely to develop side effects. They use chemotherapy to keep a young child's tumour under control. Children usually have chemotherapy every 8 weeks for about a year. 

If ependymoma comes back 

Your specialist might recommend further radiotherapy or chemotherapy to shrink your tumour. Treatments can slow down the growth of your tumour and help to reduce side effects. 

Coping with ependymoma

Coping with a diagnosis of cancer can be difficult, both practically and emotionally. It can be especially difficult if you have a rare cancer. Being well informed about your cancer and its treatment can make it easier to make decisions and cope with what happens.

Talking to other people who have the same thing can help. But it can be hard to find people who have had a rare type of cancer.

Follow up

You will have regular check ups once you finish your treatment. Your doctor will examine you and ask about your general health.

This is your chance to ask questions and to tell your doctor if anything is worrying you.

How often you have check ups depends on your individual situation.

Research and clinical trials

There may be fewer clinical trials for rare types of cancer than for more common types.

It is hard to organise and run trials for rare cancers. Getting enough patients is critical to the success of a trial. The results won't be strong enough to prove that one type of treatment is better than another if the trial is too small.

The International Rare Cancers Initiative (IRCI) aims to develop more research into new treatments for rare cancers. They are designing trials that involve several countries so that more people will be available to enter trials.