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Ependymoma

Ependymomas usually 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.

They are most common in children under 5 years old.

Around 30 children are diagnosed with ependymoma every year in the UK.

Where do ependymomas start in children?

Diagram showing the parts of the brain

In children, ependymomas most often occur in the cerebellum. They might develop in the cerebrum, or less commonly in the spinal cord. They can spread into the fluid that surrounds the brain.

Risks and causes

Most people worry about what caused their child to develop cancer. But we don’t know what causes, or how to prevent most childhood cancers, including ependymoma.

We know that there is a small increased risk of ependymoma with some inherited family cancer syndromes.

And rarely ependymoma is linked to:

  • familial adenomatous polyposis (FAP) - sometimes known as Turcot syndrome
  • spinal tumours in neurofibromatosis type 2 (NF2)

But remember, not every child with one of these syndromes develops a brain tumour.

Signs and symptoms

Your child might have symptoms for a few weeks or months before they are diagnosed with ependymoma. Many of the symptoms are general and non specific. Some are similar to less serious childhood illnesses. Symptoms include:

  • headaches in the morning
  • feeling or being sick
  • loss of balance
  • seizures
  • head tilt

Young infants might have an increasing head circumference, which is measured by a health professional during their well baby check ups. Or the soft spot on top of the skull (fontanelle) might be swollen.

Tests

The first tests your child might have are a:

  • CT scan
  • MRI scan

They might also have a lumbar puncture. This is a test to check for cancer cells in the fluid that surrounds the brain and the spine. This fluid is called cerebrospinal fluid (CSF). Your child’s doctor or specialist nurse will take a sample from the fluid around their spine.

For some types of test, including a lumbar puncture your child might need sedation or a general anaesthetic. This is to help them lie still.

Getting diagnosed

We have information for parents whose child has just been diagnosed with cancer. It has information about what to expect.

Even if you have been told that a brain tumour is a possibility, hearing this confirmed by a doctor can be shocking.

Types and grades

For most types of brain tumours usually doctors look at the cells under a microscope. This can usually give an idea if the tumour is slow or fast growing. This is called the grade. But for ependymomas the appearance 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.

Researchers are learning more about ependymoma all the time. Ependymoma is grouped by what the cells look like under a microscope.

There are a number of types of ependymoma. They are:

Grade 1

  • myxopapillary ependymoma
  • subependymoma

Grade 2 and 3

  • ependymoma which includes papillary, clear cell and tanycytic
  • RELA fusion positive ependymoma – research has recently shown this type has a gene change inside the ependymoma cells
  • anaplastic ependymoma

Decisions about treatment

Your child’s specialist team decides on the best treatment options for your child. Both you and your child will be involved in decisions about treatment and care.

The treatments are usually the same for all types of ependymoma.

The most common treatments are:

  • surgery
  • radiotherapy

Less commonly, your child might have chemotherapy.

Surgery

The exact type of surgery your child needs depends on where the ependymoma is in their brain. The surgeon will aim to remove as much of the tumour as possible during surgery.

Hearing that your child needs brain surgery (neurosurgery) can feel very scary. We have more information written for parents and carers about this type of surgery.

Radiotherapy

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

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

Very young children are more likely to develop side effects form the radiotherapy. So, these children usually have chemotherapy instead of radiotherapy after surgery.

It’s unlikely that your child will have radiotherapy if they are under 3 years and they have a tumour in the supratentorial area (the area that contains the cerebrum). Children over 18 months might have radiotherapy, if they have a tumour in an area called the posterior fossa.

Proton beam therapy

Proton beam therapy is a type of radiotherapy.

It uses protons rather than high-energy x-rays to kill cancer cells. Protons are tiny parts of atoms. They work differently from x-rays in that they produce a sudden burst of energy when they stop, which stays inside the tumour. The aim is that there is less damage to healthy cells around the tumour.

Photo of a child having proton beam therapy

Research shows that proton beam therapy can work well for some types of children’s brain tumours. This includes some, but not all, children with an ependymoma.

Your child’s specialist will talk with you if they think proton therapy might be beneficial for your child.

In 2009, the UK government made the decision to set up a National NHS Proton Beam Therapy Service. One therapy centre is open at The Christie hospital in Manchester. The other will be at University College London hospital. At the moment, some children needing this service will need to travel abroad for it.

Chemotherapy

Chemotherapy is the use of anti cancer drugs to destroy cancer cells.

Children under 3 usually have chemotherapy instead of radiotherapy after surgery. Doctors use chemotherapy to keep a young child's tumour under control. It’s likely your child will have chemotherapy every 8 weeks for about a year.

Side effects of treatment for ependymoma

The side effects of treatment are different for each child. Some side effects of chemotherapy and radiotherapy happen during treatment and stop once treatment finishes. Other side effects happen months or years after treatment. These are known as late effects or long term effects. They can be mild or more challenging. Not all children have challenging long term side effects.

To help your child with any long term side effects a team of specialists will closely monitor them after treatment finishes.

The possible side effects of radiotherapy might lead to problems at school or with your child meeting their developmental milestones.

We have detailed information on follow up and late effects of children’s brain tumour treatment, what treatments can help and where to get support.

Clinical trials

Researchers are interested in finding new and kinder ways to treat ependymoma. And ways to reduce the side effects of treatment. Many children have their treatment for a brain tumour as part of a clinical trial.

SIOP Ependymoma Program II

One trial called SIOP Ependymoma Program II is looking at radiotherapy and chemotherapy after surgery. Half the children and young people taking part will have just radiotherapy. The other half will have chemotherapy after radiotherapy.

The aims of this trial are to find out:

  • how well chemotherapy after surgery and radiotherapy works to stop ependymoma coming back
  • about the side effects of having chemotherapy after surgery and radiotherapy
  • how safe it is to give chemotherapy after surgery and radiotherapy

Coping when your child has a brain tumour

We have information about where to get help and support when your child has a brain tumour.

Last reviewed: 
13 Mar 2019
  • Incidence statistics from Cancer Research UK (CancerStats)
    Accessed July 2018

  • The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary
    DN Louis and others
    Acta Neuropathologica, 2016

  • UpToDate Ependymoma
    Last updated May 2018

  • Clinical Commissioning Policy: Proton Beam Radiotherapy (High Energy) for Teenage and Young
    Adult Cancer Treatment – NHS Overseas Programme

    NHS England, 2015

  • The Road Map for National Health Service Proton Beam Therapy
    A Crellin
    Clinical Oncology, 2018
    Volume 30

  • Cancer Research UK Clinical Trials Database
    Accessed August 2018

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