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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.
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.
More research is needed into how each of these types behave.
The signs and symptoms, that a child with an ependymoma may display, depend on a number of factors, including the age of the child, the location of the tumour and its size. An ependymoma may cause two main types of symptoms: general and focal.
Focal symptoms refer to symptoms caused specifically by a tumour within the posterior fossa (where most ependymomas are found) rather than general symptoms caused by a mass anywhere in the brain. These could be:
If your child displays one or more of the general or focal symptoms, consult a doctor who may run further diagnostic tests to determine their cause.
If your doctor suspects that your child has a brain tumour, they will refer him/her ti a specialist called a neurologist that specialises in brain and nerve disorders or an oncologist. The speciliast will ask questions about your child's health and give them a physical examination. They will also test their nervous system (called a neurological examination). This involves looking at your child's vision, hearing, alertness, muscle strength, co-ordination, and reflexes. They will also look at the back of their eyes to see if there is any swelling of the optic disc (the optic disc is where the optic nerve from the brain enters the eye). Any swelling is a sign of raised pressure inside the skull, which could be a sign of a brain tumour.
They will also need an MRI (magnetic resonance imaging) or CT (computerised tomography) scan to confirm whether a brain tumour is present. If from a scan, a tumour is identified, a resection (biopsy) will be planned to remove as much of your child's tumour as possible for diagnosis (for testing). A resection to remove a brain tumour is a complex operation which takes several hours. Any risks will be explained to you by your child's surgical team. If possible, the neurosurgeon will attempt to remove the whole tumour during the resection.
In both cases, cells from the tumour will be analysed in a laboratory by a neuropathologist. The neuropathologist will examine the cells under a microscope and make a diagnosis based on the characteristic tissue patterns of ependymoma. Also, the tumour will be divided into one of the four different sub-groups (types). It is important that a detailed diagnosis of the exact tumour type is made as this will allow your medical team to determine the best course of treatment for your child.
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, 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 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.
Page last reviewed: 04/2015
Next review due: 04/2018
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