Ependymoma

What are ependymomas?

Ependymomas develop from ependymal cells. These cells line the fluid filled areas of the brain (ventricles) and the spinal cord. Ependymal cells are a type of glial cell that support and protect nerve cells.

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

Find out more about the ventricles and the spinal cord

Symptoms of ependymomas

Symptoms of ependymomas depend on whether the tumour is in the brain or the spinal cord.

Common symptoms of an ependymoma in the brain can include:

• headaches

• feeling or being sick

• changes in eyesight

• dizziness

If the tumour is in the spinal cord, symptoms can include:

• back pain

• neck pain

• numbness or weakness in the arms or legs

Find out more about the symptoms of brain tumours

Types of ependymomas

Doctors use a system to group (classify) brain tumours into different groups and types. The World Health Organisation (WHO) regularly update this system. The information on this page is based on the latest WHO classification of 2021.

There are different types of ependymoma. The doctors base the types on:

• what the tumour cells look like under the microscope

• where the tumour is

• changes (mutations) in genes in the tumour cells

There are different types of ependymoma in adults. Some of these types are further divided into subtypes. This is based on changes to genes within the tumour. The main types are:

• posterior fossa ependymoma – these are in the lower back part of the brain (including the cerebellum and brain stem)

• supratentorial ependymoma – these are in the upper part of the brain (including the cerebrum)

• spinal cord ependymoma – these are in the spinal cord

• subependymoma – these are slow growing tumours that can develop in any part of the brain or spinal cord

• myxopapillary ependymoma – these are slow growing tumours that usually develop in the lower part of the spinal cord

Grades of ependymomas

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).

The grade depends on how the cells look. Generally, the more normal the cells look, the lower the grade. The more abnormal the cells look, the higher the grade. Grade also depends on genes and proteins in the tumour cells. 

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, 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. 

For ependymomas:

• most types are either grade 2 or grade 3

• subependymomas are grade 1

Read more about the grades of brain tumours

How common it is

Ependymomas are rare tumours. Most are grade 2 or grade 3 tumours.

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)

• a ​biopsy

• a test to check the fluid that circulates around the brain and spinal cord (lumbar puncture)​

We have more information about the tests you might have to diagnose a brain tumour

Tests to look a genetic changes

A specialist doctor looks at a sample of the tumour under a microscope. They may also do tests to look for specific genetic changes in the tumour. These tests are called biomarker tests or molecular tests.

The results can help doctors identify the type and grade of brain tumour. They can also help doctors decide which treatments might work best and how likely the tumour is to respond to treatment.

Treatment

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

Surgery

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

We have more information about surgery for brain tumours

Radiotherapy

Radiotherapy uses high energy x-rays to destroy abnormal 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 just to the area of the tumour if there are no tumour cells found in the cerebrospinal fluid. 

Find out about radiotherapy for brain tumours

If ependymoma comes back

Your specialist might recommend more radiotherapy or chemotherapy to shrink the tumour. Some people might have further surgery.

Treatments can slow down the growth of the tumour and help you feel better. 

Read more about chemotherapy for brain tumours

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.

You might have an MRI scan every 3 to 6 months, for two years. You then continue to have an MRI scan. How often depends on your situation.

Having scans can be worrying. It can help to speak to family or friends about how you feel. Or you can get in touch with your specialist nurse. You should also speak to your specialist nurse if you have any symptoms between your appointments.

Read more about follow up appointments after treatment for brain tumour

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.  

Find out what you can do, who can help and how to cope with a brain tumour