Ependymoma

Ependymoma is a rare type of brain tumour. It starts from ependymal cells in the brain or spinal cord. Ependymomas can develop in children and adults. This page is about ependymomas in 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

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 looks like under the microscope
  • where the tumour is
  • changes (mutations) in genes in the tumour cells

There are 5 main 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 5 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
Diagram showing the parts of the brain

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

How common it is

Ependymomas are rare. About 1 out of every 100 brain tumours (about 1%) diagnosed in England between 1995 and 2017 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 Open a glossary item or CT scan Open a glossary item
  • a test of your neurological system (neurological examination)
  • a biopsy Open a glossary item

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 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 to just the area of the tumour if there are no tumour cells found 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.

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. It might be every 6 months, every year or every 2 years.

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.

  • Cancer: Principles and Practice of Oncology (12th edition)
    VT DeVita, TS Lawrence, SA Rosenberg
    Wolters Kluwer, 2023

  • Brain tumours (primary) and brain metastases in adults
    The National Institute for Health and Care Excellence (NICE), July 2018

  • Updates in the classification of ependymal neoplasms: The 2021 WHO Classification and beyond
    C Kresbach and others
    Brain Pathology, 2022 Volume 32, Issue 4 edition 13068
     

  • The 2021 WHO Classification of Tumors of the Central Nervous System: a summary.
    D Louis and others
    Neuro Oncology, 2021 Volume 23, Issue 8, Pages 1231-1251

  • The incidence of major subtypes of primary brain tumors in adults in England 1995-2017
    H Wanis and others
    Neuro Oncology, 2021 Volume 23, Issue 8, Pages 1371-1382

  • EANO guidelines for the diagnosis and treatment of ependymal tumors
    R Ruda and others
    Neuro-Oncology, 2017 Volume 20, Issue 4, Pages 445-456

  • Ependymoma: Evaluation and Management Updates
    R Rudà and others
    Current Oncology Reports, 2022 Volume 24, Issue 8, Pages 985-993

Last reviewed: 
08 Jun 2023
Next review due: 
08 Jun 2026

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