Ependymoma

Ependymoma is the third most common type childhood brain tumour and is the type of glioma most commonly found in children.

While ependyomas can develop in people of any age, the average age of diagnosis is five years old and at least a quarter of diagnoses happen before the age of two. 

Most childhood ependymomas are found in the hindbrain, which is sometimes called the posterior fossa. This is the lower part of the brain which controls functions, such as balance and movement, and vital functions, such as breathing and blood pressure.

What is an ependyoma?

Ependymomas are a type of brain tumour that are formed from a type of cell in the brain called an ependymal cell. Ependymal cells are found lining the ventricles of the brain (the fluid–filled spaces in and around the brain) and their job is to produce cerebrospinal fluid (CSF).

Ependymal cells belong to a larger family of cells called glial cells. This is why your child’s ependymoma may sometimes be referred to as a glioma, which is the name for a tumour arising from glial cells.

Are there different types of ependymoma?

There are four different types of ependymoma, so you might hear your child’s doctor call the brain tumour by a more specific name. The different types are:

• Myxopapillary Ependymomas (grade 1)

This type of ependymoma is relatively uncommon in children and develops more often in the lower part of the spinal column rather than the brain.

• Subependymomas (grade 1)

Subependymomas are also relatively uncommon in children and appear most often near a ventricle.

• Ependymomas (grade 2)

Ependymomas are the most common type of ependymal tumour and usually appear close to, or in, a ventricle in the hindbrain.

• Anaplastic Ependymoma (grade 3)

Anaplastic ependymomas are the fastest growing type of ependymal tumour and also commonly grow in the hindbrain.

What are the symptoms of an ependymomas?

Most child ependymomas develop along the ventricle in the hindbrain known as the fourth ventricle. Depending on where in the fourth ventricle the tumour is located, your child might have one or more of the following symptoms:

• Their head seeming to be twisted or tilted (known as torticollis or wry neck)
• Loss of balance (known as ataxia)
• Problems speaking (known as dysarthria)
• Difficulty swallowing (known as dysphagia)
• Making overly clumsy movements (known as dysmetria).
  

They may also experience some of the general symptoms of childhood brain tumours.

How are ependymomas treated?

Surgery

The first treatment your child is likely to have is surgery. Their neurosurgeon will aim to remove as much of the tumour as possible. You might hear this surgery be called a resection. A resection to remove a brain tumour is a complex operation which takes several hours and nny risks will be explained to you by your child’s surgical team.

If the tumour is low grade and the surgeon is able to remove all the tumour, your child may not need any other treatment. However, if it’s not possible to remove all the tumour, or if the tumour is high grade, then your child’s healthcare team will advise radiotherapy to treat any tumour cells that may be left.

This is also the case if surgery is not possible at all. Sometimes the tumour is in a place in the brain that makes it too difficult to reach or it’s not safe to operate on. Radiotherapy is used instead. However, if your child is under 3 years old, radiotherapy is generally avoided. Then chemotherapy will be used instead.

Radiotherapy

Radiotherapy, or radiation treatment, uses controlled doses of high-energy beams to destroy tumour cells, while causing as little damage as possible to surrounding healthy cells.

Radiotherapy may be used where surgery isn’t possible, or more commonly, after surgery to kill any remaining ependymoma cells. This is to make the tumour less likely to recur (come back).

Radiotherapy does have some potential long-term side-effects on the brain, particularly in children as their brains are still developing. The risks and benefits for each child have to be weighed before deciding if radiotherapy should be given in any form. 

As a result, radiotherapy isn’t commonly given to children under the age of 3 as they’re more likely to develop long-term side–effects.

Chemotherapy

Chemotherapy is a treatment which involves drugs that have been developed to kill tumour cells. It is generally only used to treat ependymomas in children who cannot have radiotherapy. It is rarely used to treat adults with ependymoma.

Chemotherapy drugs can be taken orally (by mouth). This can be as a pill, but can be given as a liquid if your child is too young to swallow pills. Chemotherapy can also be given intravenously (into a vein). The treatment cycle is usually spread over a set period of time.

There’s ongoing research on the possible benefits of different combinations of chemotherapy drugs and radiotherapy in treating childhood ependymomas. Your oncologist may offer your child the chance to take part in a research study, which are also known as clinical trials.

What side–effects can treatment cause?

The symptoms that you may have seen in your child before treatment can be known to worsen after surgery. Other side-effects of brain surgery include headaches and feeling sick and tired.

Radiotherapy and chemotherapy also have their own side–effects. These can include hair loss, tiredness and a reduced appetite. If you’re worried about your child after treatment, speak to your child’s oncologist for advice and help with treating symptoms.

Hearing that your child may have long–term side–effects, due to treatment, might be hard to hear. But your child’s healthcare team will have taken these into consideration and will only give treatment where the benefits outweigh the risks.