What is Ependymoma?

Ependymomas are a type of tumour that arise from the cells that line the brainís fluid spaces, known as the ventricles. These represent roughly 10% of all central nervous system (brain and spine) tumours in children. Approximately 60% of all children diagnosed with this tumour type are less than 5 years of age. As with other brain tumours in children, the cause of these tumours remains unknown.

Signs & Symptoms

Ependymomas are twice as likely to occur in the posterior fossa region of the brain. This is the area at the lower back of the head, above the neck. When tumours arise in this area, children commonly present with signs and symptoms of increased intracranial pressure (increased pressure within the cranium or skull). This is because the tumour is sitting and obstructing, or blocking off the normal pathway of cerebrospinal fluid (the fluid that surrounds the brain and spine). This results in a build up of fluid in the brain, resulting in an increase in pressure. Children may complain of headaches, vomit or have an unsteady walk as a result of this pressure increase.

Although less common, these tumours may also arise in the top part of the brain, known as the cerebral hemispheres. When tumours arise in these areas, children may experience headaches, visual disturbances and possibly seizures.

Diagnosis

The diagnosis of an Ependymoma, like other brain tumours, may be made by an MRI (Magnetic Resonance Imaging) scan, however a biopsy is necessary to confirm this. An MRI of the brain is performed prior to surgery, so that the exact location of the tumour is determined. Other tests and investigations that help assist with staging of the disease include a lumbar puncture (or spinal tap). This detects the presence of tumour cells in the cerebro-spinal fluid (Refer to section on tests and investigations).

Treatment

Tumours that arise in the posterior fossa (lower back of the head) are often more difficult for the Neurosurgeon to completely remove compared to those in the top areas of the brain. This is because they have the tendency to infiltrate or spread into a vital part of the brain known as the brain stem. This is a delicate area of the brain that must be preserved and remain uninterrupted by the Neurosurgeon.

Following surgery to the posterior fossa region, children over the age of four will receive localised radiotherapy to the original location of the tumour and a small margin surrounding it. This is necessary to kill any microscopic tumour cells that remain in the tissue surrounding the original tumour site, including those that may remain around the brain stem. These cells will not be visible on the post-operative MRI scan. These children do not require further treatment following radiotherapy, only frequent MRI scans to monitor for recurrence. Children, who have had an incomplete or partial removal of their tumour at the initial surgery, will receive a course of chemotherapy following radiation treatment.

When Ependymomaís arise in the top part of the brain, the Neurosurgeon is generally able to remove the entire tumour without causing harm to the healthy surrounding tissue. In this group, no additional therapy (ie chemotherapy and radiotherapy) is performed, and the child undergoes regular MRI scans to monitor for recurrence of the disease.

Follow Up Care

After treatment has finished, children visit clinic on a regular basis, and undergo MRI scans and other investigations throughout their childhood to detect any possible recurrence. Importantly, all children attend the Neuro-Oncology Long-Term Effects clinic, where they are regularly reviewed a by a group of specialists involved in monitoring the specific long term growth and development needs of these patients.