Ependymoma Ependymal tumors begin in the ependyma, cells that line the passageways in the brain where cerebrospinal

fluid (CSF) is produced and stored. Ependymomas are classified as either supratentorial (in the cerebral hemispheres) or infratentorial (in the back of the brain). Variations of this tumor type include subependymoma, subependymal giant-cell astrocytoma, and malignant ependymoma. Ependymoblastoma, which occurs in infants and children under three years, is no longer considered a subtype of ependymoma. For ependymoblastoma, see primitive neuroectodermal tumor (PNET) in the Non-glial Tumors section.

Characteristics

Usually localized to one area of the brain Develops from cells that line the hollow cavities at the bottom of the brain and the canal containing the spinal cord Can be slow growing or fast growing May be located in the ventricles (cavities in the center of the brain) May block the ventricles, causing hydrocephalus (water on the brain) Sometimes extends to the spinal cord Common in children, and among men and women in their 40s and 50s Occurrence peaks at age five and again at age 34 Accounts for two percent of all brain tumors

Symptoms

Severe headaches Nausea and vomiting Difficulty walking Fatigue and sleepiness Problems with coordination Neck pain or stiffness Visual problems

Treatment
The doctor will perform tests to determine if it has spread to the spinal cord. Surgery followed by radiation therapy is the usual course of treatment. A shunt may be needed to treat hydrocephalus caused by blockage of the ventricles.

Additional Resources

Visit our Online Educational Programs page to view past webinars on ependymomas. Engage in discussion in our ependymoma forum. Watch videos by the Collaborative Ependymoma Research Network (CERN) Foundation.

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 childs 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 tumors 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 childs 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 childs physician may recommend radiation to the whole brain and spinal cord.

Chemotherapy Chemotherapy is a group of drugs that interfere with the cancer cells 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? Weve 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 childs 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 Childrens. 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 Childrens. Faith-based support: If you are in need of spiritual support, we will help connect you with the Childrens 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 childs 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, DanaFarber/Children's Hospital Cancer Care established the Stop & Shop Family Pediatric NeuroOncology 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.