Brain stem glioma begins when cells in the brain stem begin to grow uncontrollably and form a mass, called a tumor. A glioma is a tumor that grows from a glial cell, which is a supportive cell in the brain. A tumor can be malignant (cancerous), which means that it can invade other tissue and spread to other areas of the body, or it can be benign (noncancerous).
The brain stem connects the brain to the spinal cord. It is the lowest portion of the brain, located above the back of the neck. The brain stem controls many of the body’s basic functions, such as motor skills, sensory activity, coordination and walking, the beating of the heart, and breathing. It has three parts:
The midbrain, which develops from the middle of the brain
The medulla oblongata, which connects to the spinal cord
The pons, which is located between the medulla oblongata and the midbrain
Brain stem glioma is most often diffused (spread freely) through the brain stem by the time of diagnosis. This type of tumor is typically very aggressive. A small percentage of brain stem tumors are very localized or focal tumors. A focal tumor is often a low-grade (the tumor cells look similar to normal) tumor that is less aggressive.
Brain stem glioma occurs most commonly in children between five and 10 years old. Most brain stem tumors develop in the pons and grow in a part of the brain stem where it can be difficult to perform surgery, making brain stem glioma challenging to treat.
Statistics
Approximately 3,400 central nervous system (CNS; brain and spinal cord) tumors are diagnosed each year in children under the age of 20. About 850 of these are considered benign tumors. Central nervous system tumors are the second most common childhood cancer after leukemia and brain tumors.
Cancer statistics should be interpreted with caution. 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 a brain stem glioma.
Statistics adapted from the American Cancer Society's publication, Cancer Facts and Figures 2009 and the 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 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.
Doctors and researchers don’t know what causes most childhood cancers. Some evidence indicates that genetic factors may play a role in a small percentage of brain stem gliomas. Genetic conditions associated with a higher risk of developing a CNS tumor include Li-Fraumeni syndrome, tuberous sclerosis, nevoid basal cell carcinoma syndrome, and Turcot syndrome.
Children with brain stem glioma may experience the following symptoms. Sometimes, children with brain stem glioma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a brain stem glioma. If you are concerned about a symptom on this list, please talk with your child’s doctor.
Double vision or an inability to close the eyelids
Drooping of the face
Difficulty chewing and swallowing food
Weakness in the arms and legs, clumsiness or wobbliness, and difficulty walking
Doctors use many tests to diagnose a brain stem glioma and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis. However, biopsies are rarely used and are often specifically avoided in children with diffuse brain stem glioma because the results of the biopsy do not influence treatment, and they can carry major risks. In addition, the diagnosis can often be made by magnetic resonance imaging (MRI) alone (see below). Diffuse brain stem glioma is unlike most other types of tumors in this regard. For a focal tumor, a biopsy and surgical tumor removal may be considered. 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 cancer has metastasized. Your child’s 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 brain stem glioma:
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. For a brain stem glioma, this test generally does not provide enough information to make a definite diagnosis, and an MRI is required (see below).
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.
Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. A biopsy is generally not done for the more common diffuse type of brain stem tumor. However, for a focal tumor, it is often used to determine the type of tumor. If possible, a neurosurgeon (a specialist who operates on the brain and spine) will remove a small piece of tissue from the brain. 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 cancer, 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 cancer'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 cancer.
There is no formal staging system for childhood brain stem glioma. A tumor may be classified as either diffuse or focal. In addition, the tumor may be classified by its grade, which is a measure of how much the tumor cells appear like normal cells. Low-grade tumors are less aggressive than high-grade tumors.
Diffuse brain stem glioma. This type of tumor spreads freely throughout the pons and often spreads to the midbrain, the medulla, or nearby parts of the brain. These tend to be high-grade tumors; they are very aggressive and contain abnormal-looking cells.
Focal brain stem glioma. About 20% of brain stem tumors are focal, meaning they occur at one point or are contained within a small segment of the brain stem. They usually occur in the midbrain or medulla, rather than the pons. These are usually benign or low-grade tumors; they are less aggressive and the tumor cells look fairly normal.
Recurrent brain stem glioma: Recurrent brain stem glioma is a tumor that comes back after treatment.
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) with 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 brain stem glioma depends on the size and location of the tumor, whether the cancer has spread, and the child’s overall health.Three types of treatments are used to treat brain stem glioma in children: radiation therapy, chemotherapy, and surgery. Sometimes, the treatments are used in combination.
Radiation therapy
Radiation therapy is the most common treatment for brain stem glioma. Radiation therapy is the use of high-energy x-rays or other particles to kill tumor 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 therapy is given using implants, it is called internal radiation therapy or brachytherapy.
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. Because radiation therapy can sometimes interfere with the normal growth and development of the child’s brain, the doctor may choose to treat the cancer in another way. To avoid or reduce the need for radiation treatment in young children, the doctor may first use chemotherapy to shrink the tumor.
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 not an effective treatment for brain stem glioma; however, in some cases, a doctor may use chemotherapy at the same time as or after radiation therapy. 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 prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Surgery
Surgery is used to treat brain stem glioma only when the tumor’s appearance on an MRI scan is focal, which means that it may be possible to remove the cancer without damaging the brain, such as when a tumor grows out from the brain stem. For most children with diffuse types of brain stem glioma, surgery is not recommended or possible because of the location of the tumor and the risk involved.
Recurrent brain stem glioma
Treatment for recurrent brain stem glioma depends on two factors:
The type of tumor (diffuse or focal)
The type of treatment the child received for the original tumor
Depending on individual circumstances, the doctor may recommend either surgery or chemotherapy.
Doctors and scientists are always looking for better ways to treat patients with brain stem glioma. 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. Patients 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 people, 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 brain stem glioma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with brain stem glioma.
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 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 from 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.
Cancer 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 cancer 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 brain stem glioma, 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 type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the 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 cancer and different treatments, along with ways to prevent of 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 brain stem glioma 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 brain stem glioma should have life-long, follow-up care.
The child’s follow-up care plan and the long-term risks following treatment depend on several factors, such as the type of tumor and its location, the age of the child at diagnosis, and the type of treatment. For example, craniospinal radiation therapy can cause cognitive and endocrine symptoms over time, although the severity can vary greatly depending on the dose given and the age of the child. Similarly, the risks and potential side effects of surgery vary widely, depending on the location and characteristics of the tumor. Likewise, the risks of chemotherapy and the likelihood of secondary cancers strongly depend on the specific drugs used and the dosage. For each of these issues, it is important to discuss the specific aspects of the tumor and the options for treatment with the neurosurgeon and neuro-oncologist (a medical doctor who specializes in cancer of the central nervous system) that are involved in the child's care.
Based on the type of treatment your child received, the doctor will determine what examinations 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.
Children who have had cancer can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs.
Research for brain stem glioma 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 methods of imaging and surgery. Imaging techniques are being developed that help surgeons pinpoint the tumor’s location to reduce or prevent tissue damage to the healthy parts of the brain. For example, image-guided stereotaxis allows surgeons to visualize and operate on the brain using three-dimensional computerized outlines of the brain and the tumor. Along with specialized software, these images help guide the surgeon to the tumor. Tumors that were once considered inoperable are now being removed using this technique.
Improved methods of delivering radiation treatment. Conformal radiation therapy is a way to deliver high doses of radiation directly to a tumor and not healthy tissue. This technique produces detailed, three-dimensional maps of the brain and tumor, so doctors know exactly where to deliver the radiation therapy.
In addition, advances under investigation in clinical trials include drugs designed to enhance the effect of radiation therapy or to slow or stop tumor growth.
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:
What type of tumor has been diagnosed?
Where exactly is the tumor located?
Is the tumor diffuse or focal? What does this mean?
Can you explain my child’s pathology report to me?
Are other tests needed to confirm this diagnosis?
What is your familiarity with my child’s tumor type and its treatment?
What are the treatment options?
What clinical trials are open to my child?
What treatment plan do you recommend? Why?
What are the chances for success with the planned therapy?
Who is part of the treatment team, and what does each team member do?
What are the possible side effects of this treatment, both in the short term and 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 my child? 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
National Brain Tumor Society
22 Battery St., Ste. 612
San Francisco, CA 94111
Toll Free: 800-934-2873 www.braintumor.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 (800-242-4453)
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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