• Ependymoma

    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.

    Ependymoma Treatment at Dana-Farber/Boston Children's

    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:

    • receive care from neuro-oncologists, neurosurgeons, neurologists, and pediatric subspecialists.
    • have access to technological advances such as the intra-operative MRI, which allows pediatric neurosurgeons to see the tumor as they operate and remove as much of it as possible.
    • receive expert diagnosis by neuropathologists who use advanced molecular diagnostic testing.

    How are ependymomas classified?

    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
    • Anaplastic
    • Myxopapillary ependymoma

    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.

    What are the symptoms and causes of ependymomas?

    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 concerns.

    Ependymoma symptoms are most often associated with increased pressure in the brain and can include:

    • Headache, generally upon awakening in the morning
    • Hydrocephalus – buildup of fluid in the brain, which can result in increased head size in infants or headaches and vomiting in older children
    • Nausea and vomiting. These symptoms are often worse in the morning and improve throughout the day
    • Lethargy
    • Irritability
    • Problems eating or walking

    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.

    How are ependymomas diagnosed?

    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 following tests:

    • A physical exam and complete medical history. A neurological function test of reflexes, muscle strength, eye and facial movement, coordination and alertness.
    • Magnetic resonance imaging (MRI), a diagnostic procedure that produces detailed images of the structures within the brain and spine. An MRI uses a combination of large magnets, radiofrequencies and a computer to analyze organs and structures within the body. No x-rays or radiation are used.
    • Computerized tomography scan (also called a CT or CAT scan), a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images, often called slices, of the body from horizontal and vertical perspectives. CT scans are more detailed than general x-rays.
    • Biopsy, a tissue sample from the tumor obtained during a surgical procedure.
    • Lumbar puncture (spinal tap), to remove a small sample of cerebrospinal fluid (CSF). This can determine if any tumor cells have spread. This procedure can safely be performed under sedation.

    There 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.

    What are the treatments for ependymomas?

    Your child's 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:

    • Neurosurgery: Surgery is almost always the first step in treating ependymomas. Surgery is usually performed first in order to remove as much of the tumor as possible. Sometimes the tumor's location or other factors may make it impossible for pediatric neurosurgeons to remove it completely. Tumor specimens are examined by neuropathologists to determine the exact diagnosis. Your child's physician will use this information to plan the next phase of treatment.
    • Endoscopic third ventriculostomy (ETV) or ventriculo-peritoneal shunt (VP shunt): When a tumor causes blockage of cerebral spinal fluid (CSF) flow, our surgeons may perform one of two procedures in order to relieve symptoms of hydrocephalus, the build up of fluid inside the skull. In an endoscopic third ventriculostomy, neurosurgeons create a small hole that allows fluid to flow around the blockage. About 90 percent of children with symptoms of hydrocephalus will receive this procedure. In some cases, children may have an alternative procedure in which a tube is placed in the ventricles to the abdomen to drain excess fluid into the abdomen. This is called a VP shunt.
    • Radiation: High-energy waves from a specialized machine damage or shrink tumors. Precisely targeted and dosed radiation therapy is used to kill cancer cells left behind after surgery. Your child may receive this treatment in order to control the local growth of the tumor. If an ependymoma has spread, your child's physician may recommend radiation to the whole brain and spinal cord.
    • Chemotherapy: Chemotherapy are drugs that interfere with the cancer cells' ability to grow or reproduce, and may be used before surgery, to shrink the tumor. Different groups of chemotherapy drugs work in different ways and are generally systemic treatments. Your child may receive chemotherapy orally, as a pill to swallow; intramuscularly, as an injection into the muscle or fat tissue; intravenously, as a direct injection into the bloodstream, or IV; or intrathecally, as a direct injection into the spinal column through a needle.

    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.

    What is the latest research on ependymomas?

    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:

    • The role of anti-tumor medications in treating brain tumors. New therapies are currently under investigation.
    • Genetic markers on tumor cells that may help predict how well children will do with treatment and how effective different treatment strategies may be.
    • The long-term effects of treatment and ways to minimize these effects

    It’s possible 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.

    Clinical Trials

    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 clinicaltrials@danafarberbostonchildrens.org. We can help you navigate your options.

    What is the long-term outlook for ependymomas?

    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 to cure.

    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.

    Children 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.

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