Ependymoma is a rare type of brain tumour called a glioma. They start from ependymal cells in the brain or spinal cord. Ependymomas can develop in children and adults.

Symptoms of ependymomas depend on whether the tumour is in the brain or the spinal cord. Common symptoms include headaches and feeling or being sick.  

What are ependymomas?

Ependymomas develop from ependymal cell. These cells line the fluid filled areas of the brain (ventricles) and the spinal cord.

In adults, most ependymomas start in the ependymal cells that line the spinal cord. They can spread into the fluid that surrounds the brain.

Types of ependymomas

Ependymomas are grouped based on how the cells look under a microscope. There are a number of types of ependymomas. They are:

• subependymoma
• myxopapillary
• ependymomas (which can further be divided into papillary, clear cell and tanycytic)
• anaplastic

Research has recently shown there is also a type of ependymoma that has a gene change. This is called RELA fusion-positive ependymoma.  

Grades

Ependymomas are also put into groups according to how quickly they are likely to grow. This is called the grade. These tumours can be low grade (slow growing) or high grade (fast growing).

For most types of brain tumours, doctors can usually have an idea of how the tumour might behave by looking at the grade. But for ependymomas, how the cells look under the microscope (the grade) 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. 

How common it is

Ependymomas are rare. About 2 out of every 100 brain tumours (2%) diagnosed in England between 2006 and 2010 were ependymomas. 

Most ependymomas are high grade (fast growing).

What tests will I have?

You have tests to diagnose an ependymoma. Your doctor checks the size of the tumour and its location. This helps your doctor plan your treatment. The tests you might have include:

• MRI scan or CT scan
• a test of your neurological system (neurological examination)
• blood tests
• a biopsy

Treatment

Surgery is the main treatment for ependymomas. You may also have radiotherapy and chemotherapy.

Surgery

The exact type of surgery you have depends on where the tumour is. A highly specialist doctor (neurosurgeon) removes as much of the tumour as possible.

Radiotherapy

Radiotherapy uses high energy x-rays to destroy cancer cells. You might have radiotherapy after surgery to reduce the risk of the tumour coming back.

You usually have radiotherapy to the whole brain and spinal cord if the ependymoma has spread to the fluid around the brain (cerebrospinal fluid). You might have radiotherapy to just the area of the tumour if there are no cancer cells in the cerebrospinal fluid. 

If ependymoma comes back

Your specialist might recommend more radiotherapy or chemotherapy to shrink the tumour. Treatments can slow down the growth of the tumour and help you feel better. 

Follow up

You have regular appointments with your doctor or nurse after treatment finishes. Your doctor examines you at each appointment. They ask how you are feeling, whether you have had any symptoms or side effects, and if you are worried about anything. You might also have MRI scans on some visits.

How often you have checks ups depend on your individual situation.

For a low grade ependymoma, you might have an MRI scan every 6 months, for two years. You then have an MRI scan every year or every 2 years.

For a high grade ependymoma, you might have an MRI scan every 3 to 6 months, for two years. You then have an MRI scan every 6 months to every year.

Coping with ependymoma

Coping with a diagnosis of a brain tumour can be difficult, both practically and emotionally. It can be especially difficult when you have a high grade tumour. Being well informed about the type of tumour you have, and its treatment can make it easier to cope.  

Research and clinical trials

Doctors are always trying to improve the diagnosis and treatment of brain tumours. As part of your treatment, your doctor might ask you to take part in a clinical trial. This might be to test a new treatment or look at different combinations of existing treatments.