KidsMD Health Topics

Ependymoma


  • Having a tumor in the brain or spinal cord is always a very serious matter. However, there are many distinct types of brain tumors that occur in children, and some types are highly curable. Ependymomas fall in the middle the spectrum, with a cure rate of about 50 percent depending on the specific type.

    An ependymoma is tumor that comes from the cells lining the ventricular system of the brain or spinal cord, which contains cerebrospinal fluid (CSF). It is the third most common brain tumor in children.

    Ependymomas in children usually appear in the brain, most commonly in the fourth ventricle. In rare cases it is found in the spinal cord.

    • Ependymoma is treated with surgery, and, if necessary, radiation therapy.
    • Spinal cord ependymomas are often easier to treat, and have an excellent prognosis.
    • Metastatic, progressive or recurrent ependymoma is more difficult to cure.

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches ependymomas

    Children diagnosed with ependymoma are treated through Dana-Farber/Boston Children’s Cancer and Blood Disorders Center's Pediatric Brain Tumors Program. Our pediatric brain tumor specialists at work together to provide the best care possible to your child. We offer:

    • technological advances such as the intra-operative MRI, which allows our pediatric neurosurgeons to see the tumor as they operate and remove as much of it as possible
    • expert diagnosis and consultation by neuropathologists who use advanced molecular diagnostic testing
    • access to unique Phase I clinical trials, from our own investigators, the Children’s Oncology Group and the Pediatric Oncology Experimental Therapeutics Consortium

     

  • What is an ependymoma?

    An ependymoma is a tumor that comes from the cells lining the ventricular system of the brain or spinal cord, which contains cerebrospinal fluid (CSF). If your child has been diagnosed with an ependymoma, there are a number of things that you should know:

    Ependymomas account for 5 to 10 percent of pediatric brain tumors and occur equally in boys and girls.

    • The average age at diagnosis happens when a child is 5 years old, and 25 to 40 percent of patients are younger than 2.
    • In children it usually occurs in the brain, most commonly in a space called the fourth ventricle.
    • In rare cases, ependymoma may be found in the spinal cord.
    • Though ependymoma rarely occurs in the spinal cord, it does account for about 25 percent of all spinal cord tumors. Most patients with tumors of the spinal cord are older than 12.

    As you read further, you will find general information about ependymomas. If you would like to view summary information about brain tumors first, see the overview on brain tumors.

    What are the symptoms of an ependymoma?

    Your child may experience different symptoms depending on they size and location of tumor. This kind of brain tumor can block the normal flow of cerebrospinal fluid, the colorless fluid that surrounds the brain and spinal cord. This can cause increased pressure on the brain and enlargement of the skull (hydrocephalus) as well as a variety of symptoms.

    Common symptoms of ependymoma may include:

    • headaches (especially upon awakening)
    • nausea and vomiting (especially upon awakening)
    • lethargy and irritability
    • problems eating or walking
    • in infants, enlarged head size or fontanelles (the soft “spot” that occurs before the bones in the head fuse)

    The symptoms of a brain tumor may resemble other, more common conditions or medical problems. Always consult your child's physician for a diagnosis.

    What are the different kinds of ependymoma?

    Your child’s physician will perform tests to determine what type of ependymoma your child has. Ependymomas can appear differently when evaluated under the microscope, and they can be further classified as standard or anaplastic ependymoma. These two types are treated the same way and have similar prognoses.

    A third type, which occurs at the base of the spine, called myxopapillary ependymoma, tends to be much less serious than the standard and anaplastic forms.

    Do ependymomas spread to other areas of the body?

    Yes, ependymomas can metastasize, meaning they can spread into nerby 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.
    • In these situations, more extensive treatment is necessary to cure the disease.

    GLOSSARY

  • How is an ependymoma diagnosed? 

    Your child’s physician will perform a variety of diagnostic procedures to determine the exact type of tumor and whether the tumor has spread. These may include:

    • physical exam - including neurologic function tests including: reflexes, muscle strength, eye and mouth movement, coordination and alertness.

    • magnetic resonance imaging (MRI) - to produce detailed images of organs and structures within the body and/or spine

    • computerized tomography scan (also called a CT or CAT scan) – used to capture a detailed view of the body, in some cases

    • biopsy – a tissue sample taken from the tumor during a surgical procedure to provide information about the type of tumor

    • lumbar puncture (also called an LP or spinal tap) - to remove a small sample of cerebrospinal fluid (CSF) and determine if any tumor cells have spread

  • What are the treatments for an ependymoma? 

    Specific treatment to cure ependymoma will be determined by your child's physician based on: 

    • type, location, and size of the tumor
    • extent of the disease
    • your child's age, overall health, and medical history
    • how your child's doctors expect the disease to progress
    • your child's tolerance for specific medications, procedures, or therapies
    • your preference and judgment

    What are the treatments for an ependymoma?

    There are a number of treatments that your child's physician may recommend. Some of them help to treat the tumor while others are intended to complications of the disease or side effects of the treatment.

    Treatment approaches include:

    Surgery

    Surgery is usually performed first in order to remove as much of the tumor as possible. One of our experienced pediatric neurosurgeons will perform the operation and remove as much of the tumor as possible.

    • Complete removal of the tumor is ideal when 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 determine the next stages 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 and into the spinal column. 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 installed to drain excess fluid into the abdomen (VP shunt).

    Radiation therapy

    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 is a group of drugs that interfere with the cancer cell's ability to grow or reproduce. Chemotherapy before surgery may help shrink the tumor, making it possible to remove. The role of anti-tumor medications in treating ependymoma is actively being studied. New therapies are currently under investigation.

    While chemotherapy can be quite effective in treating certain cancers, the drugs cannot differentiate healthy cells from cancer cells. Because of this, there can be side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring.

    Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Your child may be given chemotherapy:

    • orally, as a pill to swallow
    • intramuscularly, as an injection into the muscle or fat tissue
    • intravenously, directly to the bloodstream (also called IV)
    • intrathecally, directly delivering the chemotherapy into the spinal column with a needle

    What is the expected outcome (prognosis) after treatment?

    Ependymoma of the brain treated with complete tumor removal and radiation therapy to the tumor area can result in about a 50 percent cure rate. Spinal cord ependymomas are often easier to treat, and have an excellent prognosis. Metastatic, progressive or recurrent ependymomas are more difficult to cure.

    Within the brain and posterior fossa, outcomes are best when the tumor can be completely removed surgically, and if high dose focal radiation therapy is given. For spinal cord ependymomas, a similar approach is used, sometimes with lower radiation doses. An ependymoma that has spread, or metastasized, may require additional whole brain and spine radiation therapy, depending on the age of your child.

    What is the recommended long-term care my child after treatment? 

    Children treated for an ependymoma should visit a survivorship clinic every year:

    • to manage disease complications
    • to be screened for early recurrence of tumor
    • to manage late effects of treatment

    A typical follow-up visit may include some or all of the following:

    • a physical exam, including neurologic exam
    • laboratory testing
    • MRI imaging scans

    Through the Stop and Shop Neuro-Oncology Outcomes Clinic as Dana-Farber Cancer Institute, your child will meet with their pediatric neuro-oncologist and neurologist at the same follow-up visit.

    • Endocrinologists, neuro-psychologists, alternative/complementary therapy specialists, and 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 treatments.

    Coping & support

    We understand that you may have a lot of questions if your child is diagnosed with a brain tumor. Will it affect my child long term? What do we do next? We've tried to provide some answers to those questions in the following pages, but there are also a number of other resources to help you and your family through this difficult time.
     
    Patient education: From the first visit through follow-up care, our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have — How long will my child be in the hospital? How often will my child require follow-up? They will also reach out to you by phone, continuing the care and support you received while at Children's.
     
    Parent to parent: Want to talk with someone whose child has been treated for a pediatric brain tumor? We can put you in touch with other families who have been through the same experience that you and your child are facing, and share with you their experience at Children's.
     
    Faith-based support: If you are in need of spiritual support, we will help connect you with the Children's chaplaincy. Our program includes nearly a dozen clergy representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
     
    Social work: Our clinical social workers have helped many other families in your situation. Your social worker can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

    Click here for more information on our pediatric support services.

    Long-term follow-up

    The majority of children and adolescents diagnosed with pediatric brain tumors will survive into adulthood. However, many of them will face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.

    To address the needs of this growing community of brain tumor survivors, Dana-Farber/Boston Children’s Cancer and Blood Disorders Center established the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic.

    Today, more than 900 pediatric brain tumor survivors of all ages are followed by the Outcomes Clinic, a multi-disciplinary program designed to address long-term health and social issues for families and survivors of childhood brain tumors. Some of the post-treatment services provided by the Outcomes Clinic include:

    • MRI scans to monitor for tumor recurrences
    • intellectual function evaluation
    • endocrine evaluation and treatment
    • neurologic assessment
    • psychosocial care
    • hearing, vision monitoring
    • ovarian dysfunction evaluation and treatment
    • motor function evaluation and physical therapy
    • complementary medicine

    As a result of treatment, children may experience changes in intellectual and motor function. Among several programs addressing these needs are the School Liaison and Back to School Programs, which provide individualized services to ease children's return to school and maximize their ability to learn.

    In addition to providing thorough and compassionate care, our Outcomes Clinic specialists conduct innovative survivorship research and provide continuing education for staff, patients and families.

    To learn more about our services or to schedule an appointment, please click here or call us at 617-632-2680.

  • The pediatric neurosurgeons at Children’s Hospital Boston offer the most recent technological advances, such as the intra-operative MRI, which allows them to visualize the tumor as they operate and remove as much of it as possible.

    We also have access to high-tech imaging, such as PET , CT and functional MRI, which enable us to understand exactly where the tumor tissue is, and to map out surgeries and treatments that minimize risk to healthy brain tissue.

    In addition, Children’s also offers stereotaxic radiosurgery (SRS) when appropriate. SRS is a form of targeted radiation therapy and is not true surgery. The therapy uses a highly focus and targeted beam of energy to deliver radiation to a very small area, sparing healthy tissues from exposure as much as possible.

    What is the latest research on ependymoma?

    We are a member of the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with relapsed or refractory disease. We’re also the New England Phase I Center of the Children’s Oncology Group. Children with progressive or recurrent ependymomas may be eligible for experimental therapies available through these consortiums.

    Currently, our researchers are examining genetic markers on tumor cells that may help predict how well children will do with treatment and how effective different treatment strategies may be.

    For more information on current research, see the Brain Tumor Program [LINK: Main Brain Tumor Program Page].

    Clinical trials

    It’s possible that your child will be eligible to participate in one of Children’s current clinical trials. These studies are useful for a multitude of reasons: Some trials are designed to evaluate the effectiveness of a particular drug, treatment or therapy on a specific disease; others help doctors to better understand how and why certain conditions occur. At any given time, Children’s has hundreds of clinical trials underway.

    Researchers at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center are currently investigating the long-term effects of treatment and ways to reduce radiation therapy in order to minimize these effects

    To search for a cancer trial at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, go to: http://www.dana-farber.org/Research/Clinical-Trials/Clinical-Trials-by-Diagnosis.aspx?did=49

    To search the NIH’s list of clinical trials taking place around the world, go to: http://www.clinicaltrials.gov/ct2/search

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