Childhood ependymoma is a type of brain tumor. A brain tumor occurs when cells in the brain become abnormal and multiply without control, forming a mass of tissue. A tumor can be benign (noncancerous) or malignant (cancerous). A brain tumor is named after the cells from which it develops. Ependymoma is a rare brain tumor that starts in the ependymal cells that line the ventricles (fluid-filled spaces) in the brain and the central canal of the spinal cord. The brain and spinal column make up the central nervous system (CNS), where all vital functions are controlled, including thought, speech, and body strength.
Ependymoma belongs to a grouping of brain tumors called gliomas. A glioma is a tumor that grows from a glial cell, which is a supportive cell in the brain. Even though an ependymoma can occur in any part of the brain or spine, it most commonly occurs in the cerebrum, which is the largest part of the brain. The cerebrum is the top part of the brain and controls motor activities and talking.
Statistics
Ependymoma occurs most often in young children, accounting for approximately 7% of all childhood brain cancer.
The five-year relative survival rate (the percentage of patients who survive at least five years after the cancer is detected, excluding those who die from other diseases) of children ages 0-14 with ependymoma is approximately 51%.
Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with ependymoma. Because the survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this cancer.
Source: Central Brain Tumor Registry of the United States.
A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors that can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.
The causes of childhood ependymoma are unknown. There are no known risk factors or ways to prevent the disease.
Children with ependymoma may experience the following symptoms. Sometimes, children with ependymoma do not show any of these symptoms. Or, these symptoms may be cause by a medical condition that is not a tumor. If you are concerned about a symptom on this list, please talk to your child’s doctor.
Doctors use many tests to diagnose a tumor and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most tumors, a biopsy is the only way to make a definitive diagnosis. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the tumor has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
The type of tumor suspected
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose ependymoma:
Computed tomography (CT or CAT) scan.A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture. A spinal MRI may be used to determine if the tumor has spread to the spine.
Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).
Staging is a way of describing a tumor, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of tumors.
There is no formal staging system for ependymoma, however, it can be classified based on the location of the tumor and if the cancer has metastasized.
Supratentorial: The tumor is above the membrane that covers the cerebellum, known as the tentorium cerebella.
Infratentorial: The tumor is growing below the tentorium cerebella.
Recurrent: A recurrent tumor is a tumor that comes back after treatment.
A tumor may also be described by its grade, which is used to describe how much the tumor appears like normal tissue when viewed with a microscope. In a tumor that resembles normal tissue, doctors can clearly see different types of cells grouped together (called well differentiated). In a higher-grade tumor, the cells usually look less like normal cells, or “wilder” (called poorly differentiated or undifferentiated). In general, a better differentiated, lower grade tumor has a better prognosis.
Clinical trials are the standard of care for the treatment of children with cancer. In fact, more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best treatments available) to newer treatments that may be more effective. Cancer in children is rare, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track progress.
To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. Many times, a team of doctors treats a child with cancer. Pediatric cancer centers often have extra support services for children and their families, such as nutritionists, social workers, and counselors. Special activities for kids with cancer may also be available.
The treatment of ependymoma depends on the size and location of the tumor, whether the tumor has spread, and the child’s overall health.
Surgery
Surgery is usually the first treatment for ependymoma. It is also used to definitively diagnose ependymoma. The goal of surgery is to remove as much of the tumor as possible. Sometimes, surgery cannot be performed because the tumor is located in a place that the surgeon cannot reach or is near a vital structure.
After surgery, a treatment plan will be devised based on the following criteria:
The amount of tumor removed
The age of the chil
d
Whether there are metastases
Radiation therapy
Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy.
Radiation therapy may be used after surgery to treat ependymoma. If the tumor has not spread, radiation therapy will be delivered to the tumor. If the tumor has spread, radiation therapy may be directed to the whole brain and the spine. It may also be used to treat a tumor that cannot be treated with surgery. Even though radiation therapy is not standard treatment for children younger than three years old, there are clinical trials evaluating its usefulness in this age group for this type of tumor.
Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy alone is often not effective to treat children with ependymoma, but it may be beneficial if used in combination with other therapies. Clinical trials combining postoperative (after surgery) radiation therapy and chemotherapy are underway. The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.
The medications used to treat cancer are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your child’s prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Doctors and scientists are always looking for better ways to treat patients with ependymoma. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. People who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.
People decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, people are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other people volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating ependymoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with ependymoma.
In order to join a clinical trial, people must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different than the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.
A tumor and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.
Fear of treatment side effects is common after a diagnosis of ependymoma, but it may be helpful to know that preventing and controlling side effects is a major focus of your child’s health-care team. Before treatment begins, talk with your child’s doctor about possible side effects of the specific treatments your child will be receiving. The specific side effects that can occur depend on a variety of factors, including the size and location of the tumor, the individual treatment plan (including the length and dosage of treatment), and your child’s overall health.
Ask your child’s doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with the doctor about side effects your child experiences during and after treatment. For more information on the most common side effects of tumors and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects, based on ASCO’s curriculum.
In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient.
For more information on late effects or long-term side effects, please read the After Treatment section or talk with your child’s doctor.
After treatment for ependymoma ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and /or medical tests to monitor your child’s recovery for the coming months and years. All children treated for tumors, including ependymoma, should have life-long, follow-up care.
Specific after-treatment recommendations must be determined on a case-by-case basis because there are many factors that can affect the outcome of treatment for ependymoma, such as tumor location, whether the tumor could be removed during surgery, the need for and type of postoperative therapy, and age at the time of treatment.
The needs of a child treated for an ependymoma should be closely monitored on an ongoing basis by the health-care team. Some children may require a combination of physical therapy and occupational therapy (for neurological side effects resulting from the tumor or its treatment), speech therapy, hearing assistance, and cognitive therapy. Some children may need a tracheostomy (a surgical opening in the windpipe to help them breathe) and/or gastrostomy (a surgical opening into the stomach to enable a feeding tube to be inserted) after surgery, and others may develop hormonal impairments or cognitive problems, or may experience new problems if the tumor recurs.
MRI scans are routinely used to monitor for signs of tumor recurrence or growth. Because most tumors that recur do so during the first several years after diagnosis, scans are performed most frequently during the initial two to three years after treatment, and less frequently thereafter. However, because a tumor can recur many years after initial treatment, long-term follow-up care of all children treated for ependymoma is extremely important.
Based on the type of treatment the child received, the doctor will determine what examination and tests are needed to check for long-term side effects and the possibility of secondary cancers. Your child’s doctor can recommend the necessary screening tests. Follow-up care should also address the child’s quality of life, including any developmental or emotional concerns. Learn more about Childhood Cancer Survivorship.
The child’s family is encouraged to organize and keep a record of the child’s medical information, so that as the child enters adulthood, he or she has a clear, written history of the diagnosis and details of the treatment given. The doctor’s office can help you compile this, and it should include recommendations from the doctor about the schedule for follow-up care. This information will be valuable to doctors who care for your child during his or her lifetime.
Research for ependymoma is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your child’s doctor.
Improved radiation therapy techniques. Although the use of radiation therapy is not standard cancer treatment for children under the age of three, its usefulness and side effects are being evaluated in a clinical trial for children with ependymoma in this age group. In addition, new techniques for planning and delivering radiation therapy are also being evaluated that better target the tumor, reducing damage to healthy parts of the brain.
New combinations of chemotherapy. New types of chemotherapy and combinations of drugs are being used to shrink any tumor that remains after surgery.
Regular communication with your child’s doctor is important in making informed decisions about his or her health care. Consider asking the following questions of your child’s doctor:
Where exactly is the tumor located?
Has it spread?
Is it likely to spread?
Can you explain my child’s pathology report to me?
What is your familiarity with my child's tumor type and its treatment?
What are the available treatment options?
What clinical trials are open to my child?
What is the chance for success with each treatment option?
What treatment option do you recommend? Why?
What are the possible side effects of this treatment, both in the short term and the long term?
How will this treatment affect my child’s daily life? Will he or she be able to go to school and perform his or her usual activities?
What follow-up tests will my child need, and how often will he or she need them?
What support services are available to me? To my family?
Alliance for Childhood Cancer 1900 Duke St., Ste. 200
Alexandria, VA 22314
Phone: 703-299-1050 www.childhoodcanceralliance.org
American Brain Tumor Association
2720 River Rd.
Des Plaines, IL 60018
Phone: 847-827-9910
Toll Free: 800-886-2282 www.abta.org
Beyond the Cure
1 South Memorial Dr., Ste. 800
St. Louis, MO 63102
Toll Free: 800-5-FAMILY (532-6459) www.beyondthecure.org
The Brain Tumor Foundation
1350 Avenue of the Americas, Ste. 1200
New York, NY 10019
Phone: 212-265-2401 www.braintumorfoundation.org
The Brain Tumor Society
124 Watertown Street, Ste. 3-H
Watertown, MA 02472
Phone: 617-924-9997
Toll Free: 800-770-TBTS (8287) www.tbts.org
Candlelighters Childhood Cancer Foundation
P.O. Box 498
Kensington, MD 20895
Phone: 301-962-3520
Toll Free: 800-366-2223 www.candlelighters.org
The Childhood Brain Tumor Foundation
20312 Watkins Meadow Dr.
Germantown, MD 20876
Phone: 301-515-2900
Toll Free: 877-217-4166 www.childhoodbraintumor.org
Children's Brain Tumor Foundation 274 Madison Ave., Ste. 1004
New York, NY 10016
Toll Free: 866-228-4673 www.cbtf.org
The Children's Cause for Cancer Advocacy
1010 Wayne Ave., Ste. 770
Silver Spring, MD 20910
Phone: 301-562-2765 www.childrenscause.org
Children's Hospice International
1101 King St., Ste. 360
Alexandria, VA 22314
Toll Free: 800-24-CHILD (800-243-4453)
Phone: 703-684-0330 www.chionline.org
CureSearch
4600 East West Hwy., Ste. 600
Bethesda, MD 20814
Toll Free: 800-458-6223 www.curesearch.org
Make-A-Wish Foundation
3550 N Central Ave., Ste. 300
Phoenix, AZ 85012
Phone: 602-279-WISH (9474)
Toll Free: 800-722-WISH (9474) www.wish.org
National Brain Tumor Foundation
22 Battery St., Ste. 612
San Francisco, CA 94111
Phone: 415-834-9970
Toll Free: 800-934-CURE (2873) www.braintumor.org
National Children's Cancer Society
1 South Memorial Dr., Ste. 800
St. Louis, MO 63102
Phone: 314-241-1600
Toll Free: 800-532-6459 www.children-cancer.com
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