Childhood Ependymoma Treatment (PDQ�)

General Information About Childhood Ependymoma

Childhood ependymoma is a disease in which malignant (cancer) cells form in the tissues of the brain and spinal cord.

The brain controls vital functions such as memory and learning, the senses (hearing, sight, smell, taste, and touch), and emotion. The spinal cord is made up of bundles of nervefibers that connect the brain with nerves in most parts of the body.

About 1 in 11 childhood brain tumors are ependymomas. Although cancer is rare in children, brain tumors are the most common type of childhood cancer other than leukemia and lymphoma.

This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment of metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary.

There are many different types of brain tumors. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. See the following PDQ summaries for more information:

• Childhood Brain and Spinal Cord Tumors Treatment Overview

• Adult Brain Tumors Treatment

The central nervous system controls many important body functions.

Ependymomas most commonly form in these parts of the central nervous system (CNS):

• Cerebrum: The largest part of the brain, at the top of the head. The cerebrum controls thinking, learning, problem-solving, speech, emotions, reading, writing, and voluntary movement.

• Cerebellum: The lower, back part of the brain (near the middle of the back of the head). The cerebellum controls movement, balance, and posture.

• Brain stem: The part that connects the brain to the spinal cord, in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.

• Spinal cord: The column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. The spinal cord and membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a signal from the brain to cause muscles to move or from the skin to the brain for the sense of touch.

The cause of most childhood brain tumors is unknown.

The symptoms of childhood ependymoma vary and often depend on the child?s age and where the tumor is located.

The following symptoms and others may be caused by childhood ependymoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of these problems occur:

• Frequent headaches.

• Seizures.

• Frequent nausea and vomiting.

• Loss of balance or trouble walking.

Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma.

The following tests and procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient?s health habits and past illnesses and treatments will also be taken.

• Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person?s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.

• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

• MRI (magnetic resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into the patient through a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).

• Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column to check for cancer cells. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.

Childhood ependymoma is diagnosed and removed in surgery.

If a brain tumor is suspected, a biopsy is done by removing part of the skull and using a needle to remove a sample of the brain tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery. An MRI may be done after the tumor is removed to find out how much tumor remains.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on:

• Whether cancer cells remain after surgery.

• The type of ependymoma and whether it begins in the brain or in the spinal cord.

• The age of the child when the tumor is diagnosed.

• Whether the cancer has spread to other parts of the central nervous system, such as the meninges (membranes covering the brain) and the spinal cord.

• Whether the cancer has spread to other parts of the body, such as the bone or lung.

• Whether the tumor has just been diagnosed or has recurred (come back).

Stages of Childhood Ependymoma

The area where the tumor is found and the child?s age are used in place of a staging system to plan cancer treatment.

Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment.

There is no standard staging system for childhood ependymoma. Instead, the plan for cancer treatment after surgery depends on the following:

• Whether any cancer cells remain after surgery.

• Whether the cancer has spread to other parts of the brain or spinal cord.

• The age of the child.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

• Through tissue. Cancer invades the surrounding normal tissue.

• Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.

• Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

The information from tests and procedures done to detect (find) childhood ependymoma is used to plan cancer treatment.

Some of the tests used to detect childhood ependymoma are repeated after the tumor is removed by surgery. (See the General Information section.) This is to find out how much tumor remains after surgery. Another procedure that may be done to find out if cancer has spread is a lumbar puncture. A lumbar puncture is a procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.

Recurrent Childhood Ependymoma

Recurrent childhood ependymoma is a tumor that has recurred (come back) after it has been treated. Childhood ependymoma commonly recurs, usually at the original cancer site. The tumor may come back as long as 15 years or more after initial treatment.

Treatment Option Overview

There are different types of treatment for children with ependymoma.

Different types of treatment are available for children with ependymoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with ependymoma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors.

Treatment will be overseen by a pediatriconcologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:

• Pediatric neurosurgeon.

• Neurologist.

• Neuropathologist.

• Neuroradiologist.

• Rehabilitation specialist.

• Radiation oncologist.

• Medical oncologist.

• Endocrinologist.

• Psychologist.

Childhood brain and spinal cord tumors may cause symptoms that begin before diagnosis and continue for months or years.

Childhood brain and spinal cordtumors may cause symptoms that continue for months or years. Symptoms caused by the tumor may begin before diagnosis. Symptoms caused by treatment may begin during or right after treatment.

Some cancer treatments cause side effects months or years after treatment has ended.

These are called late effects. Late effects of cancer treatment may include the following:

• Physical problems.

• Changes in mood, feelings, thinking, learning, or memory.

• Second cancers (new types of cancer).

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).

Three types of standard treatment are used:

Surgery

Surgery is used to diagnose and treat childhood ependymoma as described in the General Information section of this summary.

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. Childhood ependymoma may be treated with fractionated radiation therapy, which divides the total dose of radiation into several smaller, equal doses delivered over a period of days.

Certain ways of giving radiation therapy can help keep radiation away from healthy tissue:

• Conformal radiation therapy uses a computer to create a 3-D picture of the tumor. The radiation beams are shaped to fit the tumor.

• Proton-beam therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged particles of matter) to kill tumor cells.

• Stereotactic radiation therapy uses a head frame attached to the skull to aim radiation beams directly at the tumor.

Radiation therapy to the brain can affect growth and development in young children and is not standard treatment for children younger than 3 years. For this reason, conformal radiation therapy and proton-beam therapy that limit damage to healthy brain tissue are being studied in infants and children with ependymoma.

Damage to the brain in young children treated for ependymoma is not always due to the effects of radiation therapy. For example, when hydrocephalus (abnormal buildup of fluid in the brain) is found at diagnosis, it is linked with lower intelligence test scores following surgery and before radiation therapy.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI Web site.

Watchful waiting

Watchful waiting is closely monitoring a patient?s condition without giving any treatment until symptoms appear or change.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment Options for Childhood Ependymoma

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Newly Diagnosed Childhood Ependymoma

Newly diagnosed childhood ependymoma is a tumor that has not been treated. The patient may have received drugs or treatment, however, to relieve symptoms caused by the tumor.

Initial treatment for newly diagnosed childhood ependymoma is usually surgery, with or without additional treatment.

After surgery, treatment depends on the age of the child, the amount of tumor that was removed, and whether cancercells have spread to other parts of the central nervous system.

When the tumor is completely removed by surgery and cancer cells have not spread within the central nervous system, treatment may include the following:

• Radiation therapy to the tumor bed (where the tumor was before it was removed), for children aged 3 years or older.

• Chemotherapy, for children younger than 3 years.

• A clinical trial of watchful waiting, for children aged 1 year or older.

• A clinical trial of conformal radiation therapy.

• A clinical trial of chemotherapy with or without radiation therapy.

When a part of the tumor remains after surgery, but cancer cells have not spread within the central nervous system, treatment may include the following:

• Second-look surgery.

• Radiation therapy to the tumor bed, for children aged 3 years or older.

• A clinical trial of chemotherapy followed by second-look surgery and conformal radiation therapy.

When cancer cells have spread within the central nervous system, treatment may include the following:

• Radiation therapy to the whole brain and spine.

• A clinical trial of radiation therapy and chemotherapy.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with newly diagnosed childhood ependymoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Recurrent Childhood Ependymoma

Treatment of recurrent childhood ependymomas may include the following:

• Surgery.

• Radiation therapy, including stereotactic radiation therapy.

• Chemotherapy, if chemotherapy was not used when the tumor was first diagnosed.

• A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood ependymoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

To Learn More About Childhood Brain Tumors

For more information from the National Cancer Institute about childhood brain tumors, see the following

• What You Need to Know About? Brain Tumors

• Pediatric Brain Tumor Consortium (PBTC)

For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:

• What You Need to Know About? Cancer

• Childhood Cancers

• CureSearch for Children's Cancer

• Late Effects of Treatment for Childhood Cancer

• Adolescents and Young Adults with Cancer

• Young People with Cancer: A Handbook for Parents

• Care for Children and Adolescents with Cancer

• Understanding Cancer Series: Cancer

• Cancer Staging

• Coping with Cancer: Supportive and Palliative Care

• Cancer Library

• Information for Survivors/Caregivers/Advocates

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Changes to This Summary (01/06/2011)

The PDQcancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Images were added to this summary.

About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

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PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).