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An ependymoma is a rare tumor in the brain or spinal cord in children. Ependymomas arise from the ependyma, the tissue that covers the inside of the ventricles of the brain and the central canal in the spinal cord. An ependymoma is caused by a fault in the development in one of the precursor cells of the support cells.

Ependymomas can be low-grade or high-grade. A high-grade tumor has a higher degree of malignancy than a low-grade one. Every year in the Netherlands an ependymoma is discovered in about 10-15 children. This brain tumor occurs in children of all ages, but most commonly in toddlers, adolescents and young adults. If the ependymoma can be removed completely and there are no metastases, then the chance of a cure is reasonable to good. However, the tumor does tend to return.

Symptoms of this disease

Initially, most children with ependymomas mainly have ambiguous symptoms. They may have headaches, nausea or vomiting. This is mainly due to the increased pressure as a result of the tumor and/or the increase in fluid in the brain. It may be that your child has been lethargic for some time, looks cross-eyed or has started to write sloppily. Swallowing, speech and balance problems may also occur. Some children become dizzy, trip over their own legs or crawl or walk differently. Very occasionally there is numbness and failure of the nerve function in legs or other parts of the body. This usually involves a tumor in the spinal cord.

How do we make the diagnosis?

In case there is suspicion of a brain tumor, an MRI scan will always have to be performed, followed by an MRI of the spinal cord if the presence of a brain tumor is confirmed. This is necessary in order to map out the possible expansion within the central nervous system. Neurosurgical surgery will always follow in order to obtain tumor tissue for diagnosis. The aim of this operation is also to remove as much tumor tissue as possible. A biopsy is only performed in exceptional cases. The pathologist will diagnose the tumor tissue. For some of these children, cerebrospinal fluid is also taken by means of a spinal puncture to check for the presence of tumor cells. This additional liquor diagnosis always takes place after the tumor has been removed. 

Treatment of ependymomas

The treatment is radical for most children and depends on the location of the ependymoma, the grading, whether or not (much) tumor tissue is left behind and the age of the child. Treatment almost always begins with surgery.

If it is possible to completely remove the ependymoma, is low-grade and is only present in the cerebrum, then the treatment is completed and will be followed by a long period of checks. If the tumor returns, your child will usually undergo surgery again and radiotherapy will be performed.

If the ependymoma cannot be removed completely, it is high-grade and/or it is present in the cerebellum, radiotherapy will always be performed after surgery. If there is a lot of tumor tissue left behind, then the operation will usually be followed by chemotherapy and, if possible, a second operation. A decision is then taken regarding the follow-up treatment, which usually consists of radiotherapy.