An ependymoma is a tumor that comes from the cells lining the ventricular
system of the brain or spinal cord. Ependymal cells make cerebrospinal fluid
(CSF). Ependymomas in children usually appear in the brain, most commonly in
the fourth ventricle (or back part of the brain). In rare cases, an ependymoma
can arise in the spinal cord.
Ependymomas account for 5 to 10 percent of pediatric brain tumors and occur
equally in boys and girls. These types of tumors are the third most common
brain tumor in children. Though ependymomas only rarely occur in the spinal cord, they
do account for about 25 percent of all spinal cord tumors. Most patients with
tumors of the spinal cord are older than 12.
Children with ependymomas are treated at Dana-Farber/Boston Children's
through our Brain Tumor Center,
a world-renowned destination for children with malignant and non-malignant brain
and spinal cord tumors. Our brain tumor specialists have extensive
expertise in treating all types of brain tumors, including ependymomas. At
Dana-Farber/Boston Children’s, patients:
Successfully treating your child’s brain tumor depends on
where the tumor is located, whether the disease has spread and what type of ependymoma
it is. Doctors at Dana-Farber/Boston Children's will classify your child's tumor
as one of the following types:
Classic and anaplastic ependymomas are treated the same way and have similar prognoses. The third type, myxopapillary ependymoma, which usually occurs at the base of the spine, tends to be much less aggressive than the standard and anaplastic forms.
Brain tumors can cause a variety of symptoms in children depending on their
size and location. Keep in mind that the symptoms of a brain tumor may resemble
other more common conditions or medical problems. It is important to consult
your child's physician if you have
Ependymoma symptoms are most often associated with increased pressure in the
brain and can include:
As a parent, you undoubtedly want to know what may have caused your
child’s tumor. It’s important to understand that these and other brain tumors
most often occur with no known cause. There’s nothing that you could have done
or avoided doing that would have prevented the tumor from developing.
The first step in treating your child is forming an accurate
and complete diagnosis. Ependymomas are most commonly diagnosed from imaging
studies and biopsy. Your child's physician will likely order some of the
may be other diagnostic tests that your doctor will discuss with you depending
on your child's individual situation. After we complete all necessary tests,
our experts meet to review and discuss what they have learned about your
child's condition. Then we will meet with you and your family to discuss the
results and outline the best possible treatment options.
physician will determine a specific course of treatment based on several
factors, including your child's age, overall health and medical history, the
type, location, and size of the tumor and the extent of the disease.
Ependymoma treatments include:
Ependymoma treatments can depend on the site of the tumor and whether it is recurrent (doesn't resolve after initial treatment)
or not. Within the brain and posterior fossa, outcomes are best when the tumor
can be completely removed surgically, and high dose focal radiation therapy is
given. For spinal cord ependymomas, a similar approach is used. An ependymoma
that has spread, or metastasized, may require additional chemotherapy and
radiation depending on the age of your child.
Clinical trials, or research studies, are ways
of evaluating new treatment approaches. Dana-Farber/Boston
Children’s is a very experienced and leading institution in developing new
treatments for brain tumors. Clinical trials are very important for children
with hard-to-treat or relapsed conditions.
Our researchers are investigating:
that your child will be eligible to participate in one of our brain tumor clinical trials.
In addition to launching our own clinical trials, we also offer trials
available through collaborative groups such as the Children's Oncology Group
(COG) and the Pacific
Neuro-oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's
Oncology Group. If your child has a progressive or recurrent tumor, she may be
eligible for a number of clinical trials available through these groups, or
from one of our independent clinical investigators.
Participation in any clinical trial is
completely voluntary. We will take care to fully explain all elements of the
treatment plan prior to the start of the trial, and you may remove your child
from the medical study at any time.
Contact us: If you’re not sure which clinical trials might be right for
your child, email us at firstname.lastname@example.org.
We can help you navigate your options.
With complete surgical removal of the tumor, there is a 65
percent cure rate for ependymoma. Myxopapillary ependymomas have a much better
prognosis. Metastatic, progressive or recurrent ependymomas are more difficult
Ependymomas can metastasize, spreading to nearby areas of the brain or, less
commonly, to distant parts of the central nervous system. In about 12 percent
of patients, the disease has spread at the time of diagnosis, and more
extensive treatment is necessary to cure the disease. When ependymomas recur,
they usually do so at the original tumor site.
treated for an ependymoma should visit a survivorship clinic yearly to manage
disease complications, screen for
recurrence and manage late treatment side effects. A typical follow-up visit is
likely to include a physical exam, laboratory testing and imaging scans.
Through our Stop & Shop
Family Pediatric Neuro-Oncology Outcomes Clinic, children are able
to meet with their neurosurgeon, radiation oncologist, pediatric
neuro-oncologist and neurologists at the same follow-up visit. Our
multidisciplinary approach and depth of expertise will give your child on-site
access to endocrinologists, neuro-psychologists and alternative/complementary
therapy specialists. School liaison and psychosocial personnel from the
pediatric brain tumor team are also available. In addition, children needing rehabilitation may meet with speech,
physical, and occupational therapists during and after their visit.
A genetic test can explain why a child or young adult developed cancer and can help predict whether he is at risk for other conditions.