Childhood Brain Stem Glioma Treatment (PDQ®)

General Information About Childhood Brain Stem Glioma

Childhood brain stem glioma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain stem.

The brain stem is the part of the brain connected to the spinal cord. It is located in the lowest part of the brain, just above the back of the neck. The brain stem is the part of the brain that controls breathing, heart rate, and nerves and muscles used in seeing, hearing, walking, talking, and eating. Most childhood brain stem gliomas are pontinegliomas, which form in a part of the brain stem called the pons. Brain tumors are the third most common type of cancer in children.

The tumors may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause symptoms and need treatment.

This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment for 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. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. See the following PDQ treatment summaries for more information:

• Childhood Brain and Spinal Cord Tumors Treatment Overview

• Adult Brain Tumors

The cause of most childhood brain tumors is unknown.

The symptoms of childhood brain stem glioma vary and often depend on the child’s age and where the tumor is located.

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

• Loss of balance and trouble walking.

• Vision and hearing problems.

• Morning headache or headache that goes away after vomiting.

• Nausea and vomiting.

• Unusual sleepiness or change in energy level.

Tests that examine the brain are used to detect (find) childhood brain stem glioma.

The following imaging tests may be used:

• 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 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).

Some childhood brain stem gliomas are diagnosed and removed in surgery.

If the tumor has not spread widely within the brain stem or has not been diagnosed by MRI, a biopsy may be 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.

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

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

• The type of brain stem glioma.

• Where the tumor is found in the brain and if it has spread within the brain stem.

• Whether or not the child has a condition called neurofibromatosis type 1.

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

Stages of Childhood Brain Stem Glioma

The plan for cancer treatment depends on whether the tumor is in one area of the brain or has spread throughout the brain.

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 brain stem glioma. Instead, the plan for cancer treatment depends on whether the tumor is diffuse (spread throughout the brain) or focal (in one area of the brain):

• Diffuse intrinsic pontine glioma is a tumor that has spread widely throughout the brain stem. A biopsy is usually not done for this type of brain stem glioma and it is not removed by surgery. A diffuse intrinsic pontine glioma is usually diagnosed using imaging studies.

• Focal or low-grade glioma is a tumor that is in one area of the brain stem. A biopsy may be done and the tumor removed during the same surgery.

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 brain stem glioma is used to plan cancer treatment.

Some of the tests used to detect childhood brain stem glioma are repeated if the tumor is removed by surgery. (See the General Information section.) This is to find out how much tumor remains after surgery and plan further treatment.

Recurrent Childhood Brain Stem Glioma

Recurrent childhood brain stem glioma is a tumor that has recurred (come back) after it has been treated. If childhood brain stem glioma recurs, it may do so many years after initial treatment. The tumor may come back in the brain or in other parts of the central nervous system.

Treatment Option Overview

There are different types of treatment for children with brain stem glioma.

Different types of treatment are available for children with brain stem glioma. 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 brain stem glioma 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:

• Neurosurgeon.

• Neuropathologist.

• Radiation oncologist.

• Neuro-oncologist.

• Neurologist.

• Rehabilitation specialist.

• Neuroradiologist.

• 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 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).

Five types of standard treatment are used:

Surgery

Surgery is used to diagnose and treat childhood brain stem glioma as discussed in the General Information section of this summary.

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.

Radiation therapy to the brain can affect growth and development in young children. Certain ways of giving radiation therapy can help keep radiation from damaging healthy tissue:

• Conformal radiation therapy is a type of radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to normal tissue around the tumor.

• Hyperfractionated radiation therapy is radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day.

The way the radiation therapy is given depends on the type and stage of the cancer being treated. Radiation therapy may be used alone or in addition to chemotherapy.

Several months after radiation therapy to the brain, imaging tests may show changes to the brain tissue. These changes may be caused by the radiation therapy or may mean the tumor is growing. It is important to be sure the tumor is growing before planning more treatment.

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 in 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.

Because radiation therapy to the brain can affect growth and brain development in young children, clinical trials are studying ways of using chemotherapy to delay or reduce the need for radiation therapy.

Cerebrospinal fluid diversion

Cerebrospinal fluid diversion is a method used to drain fluid that has built up around the brain and spinal cord. A shunt (long, thin tube) is placed in a ventricle (fluid-filled space) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt carries excess fluid away from the brain so it may be absorbed elsewhere in the body.

Cerebrospinal fluid (CSF) diversion. Extra CSF is removed from a ventricle in the brain through a shunt (tube) and is emptied into the abdomen. A valve controls the flow of CSF.

Watchful waiting

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

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.

Radiation therapy with radiosensitizers

Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells.

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 Brain Stem Glioma

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.

Untreated Childhood Brain Stem Glioma

Untreated childhood brain stem glioma is a tumor for which no treatment has been given. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Standard treatment of diffuse intrinsic pontineglioma is usually radiation therapy.

Some of the treatments being studied in clinical trials for diffuse intrinsic pontine glioma include the following:

• Radiation therapy with a radiosensitizer.

Standard treatment of focal or low-grade glioma may include the following:

• Surgery with or without radiation therapy, which may be followed by adjuvantchemotherapy.

• Cerebrospinal fluid diversion followed by watchful waiting.

Treatment of brain stem glioma in children with neurofibromatosis type 1 may be watchful waiting. The tumors are slow-growing in these children and may not need specific treatment for years.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated childhood brain stem glioma. 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 Brain Stem Glioma

Treatment of recurrent childhood brain stem glioma depends on the type of tumor, whether it comes back in the place in which it started or in another part of the brain, and the type of treatment previously given.

Standard treatment of recurrent diffuse intrinsic pontineglioma is usually palliative therapy, to relieve symptoms and improve the patient's quality of life. The patient may also be treated in a clinical trial of a new treatment.

Treatment of recurrent focal or low-grade childhood brain stem glioma may include the following:

• Surgery.

• Radiation therapy.

• Chemotherapy.

• 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 brain stem glioma. 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 (11/04/2011)

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.

Changes were made to this summary to match those made to the health professional version.

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.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

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).