What is Ependymoma?

Ependymoma is a type of glioma (a brain tumour arising from glial cells) most commonly found in children. The majority of childhood ependymomas (around 60-70%) are located in the 'posterior fossa' which is a small space in the lower part of the skull, containing the hindbrain.

Ependymomas are the third most common type childhood brain tumour. They arise from ependymal cells that are found lining the ventricles of the brain (the fluid-filled spaces in and around the brain). Two-thirds of the cerebrospinal fluid (CSF) surrounding, protecting and nourishing the brain is produced by ependymal cells.

Types of ependymoma

Like most other types of brain tumour, the World Health Organization (WHO) classifies ependymal tumours into subtypes using a numerical grading scale. The grade is determined by how the tumour cells look under a microscope.

The lower the grade of the tumour, the slower it tends to grow.

  • Myxopapillary Ependymomas (Grade 1) – This type of ependymoma is relatively uncommon in children and tends to occur in the spinal column rather than the brain.
  • Subependymomas (Grade 1) – Also relatively uncommon in children. They tend to occur near a ventricle in the brain
  • Ependymomas (Grade 2) – Ependymomas are the most common type of ependymal tumour and usually appear close to, or within, the ventricular system in the posterior fossa.
  • Anaplastic Ependymoma (Grade 3) – Anaplastic ependymomas are the fastest growing type of ependymal tumour and also commonly originate in the posterior fossa.

More research is needed into how each of these types behave.

How are ependymomas treated?

Surgery

Surgery is the first line of treatment for this type of tumour. Through surgery, the neurosurgeon will aim to remove as much of the tumour as possible. After surgery, the doctor will create a treatment plan consisting of radiotherapy, or radiotherapy with chemotherapy, based on the amount of tumour removed, the child's age and whether the disease has spread.

Radiotherapy

Radiotherapy, or radiation treatment, uses controlled doses of high energy beams to destroy tumour cells whilst causing as little damage as possible to surrounding cells. Radiotherapy may be used where surgery isn't possible, or after surgery to kill any remaining ependymoma cells. It can also be used to prevent a tumour from returning or to slow down its growth. Studies have shown that radiotherapy can be effective in managing ependymomas. However, there are short and long term risks associated with treating children with radiation, especially those younger than three years old.

Chemotherapy

Chemotherapy is a treatment which involves taking drugs that have been developed to kill tumour cells. Chemotherapy drugs are taken either orally in the form of a pill or intravenously using an injection or drip, and a treatment cycle is usually spread over a set period of time.

Results of clinical trials on the effectiveness of individual chemotherapy drugs for childhood ependymoma have been mixed, with some drugs appearing ineffective in targeting ependymomas and others reporting effectiveness in a limited percentage of cases. There is ongoing research on the possible effects of different combinations of chemotherapy drugs and radiotherapy in treating childhood ependymomas.

Get your free brain tumour information pack

Our FREE Brain Tumour Information Pack has been designed to help you through this difficult time, to guide you through the healthcare system, answer your questions, and reassure you that you're not alone so that you feel confident when discussing treatment and care options with your medical team.

Privacy notice

We will use the details you provided to send your information pack and any other resources you request and any relevant information in the future. Your details are held on our secure database and we promise never to share them with any other organisation for their marketing purposes. You can find out more in our privacy policy.

We promise to keep your data safe and you can unsubscribe at any time in the future. More information is in our Privacy Policy.