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.