Brain Stem Glioma – Childhood

About the brain stem

The image is a drawing of different parts of a child’s brain stem and surrounding structures. The cross section of the brain stem shows the 3 main parts: the midbrain, which develops from the middle of the brain; the medulla oblongata, which connects to the spinal cord; and the pons, which is located between the medulla oblongata and the midbrain. 

The brainstem connects the brain to the spinal cord. The brainstem is the lowest portion of the brain, located above the back of the neck. It controls many of the body’s basic functions, such as motor skills, sensory activity, coordination, walking, the beating of the heart, and breathing. It has 3 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

About brain stem glioma

Brain stem glioma is a type of central nervous system (CNS; brain and spinal cord) tumor. This type of tumor begins when healthy cells in the brain stem change and grow out of control, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread. A glioma is a tumor that grows from a glial cell, which is a supportive cell in the brain.

Usually, by the time brain stem glioma is diagnosed, it is most often diffuse. This means it has spread freely through the brain stem. This type of tumor is typically very aggressive, meaning that it grows and spreads quickly. A small percentage of brain stem tumors are very localized, called focal tumors. A focal tumor is often less likely to grow and spread quickly.

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 (see the Types of Treatment section).

Brain stem glioma occurs most commonly in children between the ages of 5 and 10 years old. This section covers brain stem glioma diagnosed in children. Read more about brain tumors in adults.

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• Español: Read about brain stem glioma in Spanish. Infórmase sobre el glioma de tronco encefálico en español.
• Find a cancer doctor. Search for a cancer specialist in your local area using this free database of doctors from the American Society of Clinical Oncology (ASCO).
• Cancer Terms. Learn what medical phrases and terms used in cancer care and treatment mean.

Every person is different, with different factors influencing their risk of being diagnosed with this tumor and the chance of recovery after a diagnosis. It is important to talk with your doctor about any questions you have about the general statistics provided below and what they may mean for your child individually. The original sources for these statistics are provided at the bottom of this page.

How many children and adolescents are diagnosed with brain stem glioma?

In 2023, approximately 3,920 brain and other central nervous system (CNS) tumors will be diagnosed in children ages 0 to 14 in the United States. After leukemia, CNS tumors are the second most common childhood cancer, accounting for about 26% of cases in children younger than 15.

An estimated 13% of all childhood CNS tumors are found in the brain stem. Approximately 75% of childhood brain stem gliomas are in the pons.

What is the survival rate for brain stem gliomas?

There are different types of statistics that can help doctors evaluate a person’s chance of recovery from brain stem glioma. These are called survival statistics. A specific type of survival statistic is called the relative survival rate. It is often used to predict how having a tumor may affect life expectancy. The relative survival rate looks at how likely people with brain stem glioma are to survive for a certain amount of time after their initial diagnosis or start of treatment compared to the expected survival of similar people without this tumor.

Example: Here is an example to help explain what a relative survival rate means. Please note that this is only an example and is not specific to this type of cancer. Let’s assume that the 5-year relative survival rate for a specific type of cancer is 90%. “Percent” means how many out of 100. Imagine there are 1,000 people without cancer, and based on their age and other characteristics, you expect 900 of the 1,000 to be alive in 5 years. Imagine that there are another 1,000 people similar in age and other characteristics as the first 1,000, but they all have the same specific type of cancer that has a 5-year survival rate of 90%. This means it is expected that 810 of the people with the specific cancer (90% of 900) will be alive in 5 years.

It is important to remember that statistics on the survival rates for childhood brain stem glioma are only an estimate. They cannot tell an individual person if the tumor will or will not shorten their life. Instead, these statistics describe trends in groups of people previously diagnosed with the same disease, including specific stages of the disease.

About 3 in 4 children with all types of brain tumors combined survive at least 5 years after diagnosis. The survival rates for brain stem glioma vary based on several factors, including the grade of the brain stem glioma as either diffuse or focal. Other factors include the specific location of the tumor, a child’s age and general health, and how well the treatment plan works. Talk with your child’s doctor to learn more.

Experts measure relative survival rate statistics for brain stem glioma every 5 years. This means the estimate may not reflect the results of advancements in how brain stem glioma is diagnosed or treated from the last 5 years. Talk with your child’s doctor if you have any questions about this information. Learn more about understanding statistics.

Brain Stem Glioma: Childhood Risk Factors

What are the risk factors for brain stem glioma?

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause a tumor. Some people with several known risk factors never develop a tumor, while others with no known risk factors do.

Doctors and researchers do not know what causes most childhood tumors, including brain stem glioma. There is some evidence that genetic factors may play a role in a small percentage of brain stem gliomas.

The following genetic conditions are associated with a higher risk of developing a central nervous system (CNS) tumor, but they are not specifically associated with developing a brain stem glioma:

• Li-Fraumeni syndrome
• Neurofibromatosis type 1 (NF1)
• Nevoid basal cell carcinoma syndrome
• Tuberous sclerosis complex (TSC)
• Turcot syndrome

What are the symptoms and signs of brain stem glioma?

Children with a brain stem glioma may experience one or more of the following symptoms or signs:. Symptoms are changes that you can feel in your body. Signs are changes in something measured, like taking your blood pressure or doing a lab test. Together, symptoms and signs can help describe a medical problem. Sometimes, children with a brain stem glioma do not have any of the symptoms and signs described below. Or, the cause of a symptom or sign may be a medical condition that is not a brain stem glioma.

• Double vision or not being able 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
• Difficulty talking
• Headache
• Vomiting

If you are concerned about any changes your child experiences, please talk with your child’s doctor. Your doctor will try to understand what is causing your child’s symptom(s). They may do an exam and order tests to understand the cause of the problem, which is called a diagnosis.

If brain stem glioma is diagnosed, relieving symptoms remains an important part of care and treatment. Managing symptoms may also be called “palliative and supportive care,” which is not the same as hospice care given at the end of life. You can receive palliative and supportive care at any time during cancer treatment. This type of care focuses on managing symptoms and supporting people who face serious illnesses, such as cancer. Learn more in this guide’s section on Coping with Treatment.

Be sure to talk with your child’s health care team about the symptoms your child experiences, including any new symptoms or a change in symptoms.

Doctors use many tests to find, or diagnose, a brain stem glioma. They also do tests to learn if the tumor has spread to another part of the body from where it started. If the cancer has spread, it is called metastasis. Doctors may also do tests to learn which treatments could work best.

For most types of tumors, a biopsy is the only way to make a definite diagnosis, even if other tests can suggest that a tumor is present. During a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. However, diffuse brain stem glioma is unlike most other tumors. Brain stem glioma is usually diagnosed with magnetic resonance imaging (MRI) only (see below).

In general, a biopsy is avoided in children with diffuse brain stem glioma because the results of the biopsy do not change treatment options and the procedure can have serious risks. Sometimes, a biopsy may be used in clinical trials or when a brain stem glioma has unusual features. By testing the part of the tumor removed during a biopsy, doctors may find certain molecular features that can help plan treatment. As new treatments based on this molecular information increase and the risks of a biopsy decrease, these procedures may be done more often.

For a focal brain stem glioma, a biopsy and removing the tumor with surgery may be considered. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

How brain stem glioma are diagnosed

There are different tests used for diagnosing brain stem glioma and planning your child’s care. Not all the tests described here will be used for every person. Your child’s doctor may consider these factors when choosing a diagnostic test:

• The type of tumor suspected
• Your child’s signs and symptoms
• Your child’s age and general health
• The results of earlier medical tests

In addition to a physical examination, the following tests may be used to diagnose a brain stem glioma:

• MRI. An MRI produces detailed images of the inside of the body using magnetic fields, not x-rays. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow.
• Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these pictures into a detailed, 3-dimensional image that shows any abnormalities or tumors. A CT scan can be used to measure the tumor’s size. Sometimes, a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill or liquid to swallow. For a brain stem glioma, this test generally does not provide enough information to make a definite diagnosis, and an MRI is still needed.
• Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. As explained above, 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 find out the type of tumor. If possible, a doctor called a neurosurgeon will remove a small piece of tissue from the brain. A neurosurgeon specializes in treating central nervous system (CNS) tumors using surgery. A pathologist then analyzes the sample(s). A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

Brain Stem Glioma: Childhood: Stages and Grades

What is tumor staging?

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the tumor’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor recommend the best kind of treatment, and it can help predict a patient’s prognosis, which is the chance of recovery. There are different stage descriptions for different types of tumors.

There is no formal staging system for childhood brain stem glioma. As explained in the Introduction, a brain stem glioma may be classified as either “diffuse” or “focal.” In addition, the tumor may be classified by its grade.

Grade

Grade describes how much tumor cells look like healthy cells when viewed under a microscope. The doctor compares the tumor’s tissue with healthy tissue. Healthy tissue usually contains many different types of cells grouped together. If the tumor tissue looks similar to healthy tissue and contains different cell groupings, it is called differentiated or a low-grade tumor. If the tumor tissue looks very different from healthy tissue, it is called poorly differentiated or a high-grade tumor. The tumor’s grade may help the doctor predict how quickly it will spread. In general, the lower the tumor’s grade, the better the prognosis.

Below are the general classifications for brain stem glioma:

• Diffuse brain stem glioma. This is the most common classification of 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. If a biopsy was done, a diffuse brain stem glioma may be further classified by a specific genetic change in the tumor cells called H3 K27M.
• Focal brain stem glioma. About 20% of brain stem tumors are focal, meaning they occur in 1 area or are contained within a small portion 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 more like healthy cells.
• Recurrent brain stem glioma. Recurrent brain stem glioma is a tumor that has come back after treatment. If the tumor does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

In general, tumors in children are uncommon. This means it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That is why more than 60% of children are treated as part of a clinical trial. A clinical trial is a research study that tests a new approach to treatment. The “standard of care” is the best treatments known based on previous clinical trials. Clinical trials may test such approaches as a new drug, a new combination of existing treatments, or new doses of current therapies. The health and safety of all children participating in clinical trials are closely monitored.

To take advantage of these newer treatments, children should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. For brain stem glioma, a neuro-oncologist may also be involved with treatment. A neuro-oncologist is a doctor who specializes in central nervous system (CNS) tumors. If a pediatric cancer center is not nearby, general cancer centers sometimes have pediatric specialists who are able to be part of your child’s care.

How brain stem glioma are treated

In many cases, a team of doctors works with a child and the family to provide care. This is called a multidisciplinary team. Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, dietitians, physical therapists, occupational therapists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available. Learn more about the clinicians who provide cancer care.

Treatment options and recommendations depend on several factors, including the type and grade of the tumor, possible side effects, the family’s preferences, and the child’s overall health. The main types of treatments used for brain stem glioma in children are radiation therapy, chemotherapy, and surgery. Sometimes, these treatments are used together.

The treatment of brain stem glioma for children with the genetic condition neurofibromatosis type 1 (NF1) may differ. A tumor in a child with NF1 may be low-grade, even though it looks diffuse. Therefore, an approach called “active surveillance” or “watchful waiting” may be recommended. This approach occurs when the patient is closely monitored to watch for signs that the tumor is worsening. Active treatment would begin if the tumor starts to grow and spread.

Take time to learn about all of your child’s treatment options and be sure to ask questions about things that are unclear. Talk with your child’s doctor about the goals of each treatment and what you can expect during the treatment. These types of conversations are called “shared decision-making.” Shared decision-making is when you and your child’s doctors work together to choose treatments that fit the goals of your child’s care. Shared decision-making is important for brain stem glioma because there are different treatment options. Learn more about making treatment decisions.

The common types of treatments used for brain stem glioma are described below. Your child’s care plan may also include treatment for symptoms and side effects, an important part of care. Learn more about preparing your child for treatment.

Physical, emotional, social, and financial effects of a CNS tumor

A CNS tumor and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative and supportive care. It is an important part of your child’s care that is included along with treatments intended to slow, stop, or eliminate the tumor.

Palliative and supportive care focuses on improving how your child feels during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age, type, or grade of tumor, may receive this type of care. And it often works best when it is started right after the diagnosis. People who receive palliative and supportive care along with treatment for the tumor often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. Your child may also receive palliative treatments, such as chemotherapy, surgery, or radiation therapy, to improve symptoms.

Before treatment begins, talk with your child’s doctor about the goals of each treatment in the recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative and supportive care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.

Medical care is often expensive, and navigating health insurance can be difficult. Ask your doctor or another member of your health care team about talking with a financial navigator or counselor who may be able to help with your financial concerns.

During treatment, your child’s health care team may ask you to answer questions about your child’s symptoms and side effects and to describe each problem. Be sure to tell the health care team if your child is experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative and supportive care in a separate section of this website.

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Remission and the chance of recurrence

A remission is when the tumor cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.

A remission may be temporary or permanent. This uncertainty causes many people to worry that the tumor will come back. While many remissions are permanent, it is important to talk with your child’s doctor about the possibility of the tumor returning. Understanding your child’s risk of recurrence and the treatment options may help you feel more prepared if the disease does return. Learn more about coping with the fear of recurrence.

If the tumor does return after the original treatment, it is called a recurrent tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

If a recurrence happens, a new cycle of testing will begin to learn as much as possible about it. After this testing is done, you and your child’s doctor will talk about the treatment options. Treatment for recurrent brain stem glioma depends on the type of tumor, such as whether it is diffuse or focal, and the type of treatment that was given for the original tumor. Depending on the situation, the doctor may recommend either surgery or chemotherapy. Your child’s doctor may suggest clinical trials that are studying new ways to treat a recurrent brain stem glioma. Whichever treatment plan you choose, palliative and supportive care will be important for relieving symptoms and side effects.

A recurrent tumor may bring up emotions such as disbelief or fear. You and your family are encouraged to talk with your child’s health care team about these feelings and ask about support services to help you cope. Learn more about dealing with a recurrence.

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If treatment does not work,

Although treatment is successful for many children with a CNS tumor, sometimes it is not. If a child’s tumor cannot be cured or controlled, this is called an advanced or terminal tumor. This diagnosis is stressful, and advanced brain stem glioma may be difficult to discuss. However, it is important to have open and honest conversations with your child’s health care team to express your family’s feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help.

Hospice care is designed to provide the best possible quality of life for people who are expected to live less than 6 months. Parents and guardians are encouraged to talk with the health care team about hospice options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Some children may be happier and more comfortable if they can attend school part-time or keep up with other activities and social connections. The child’s health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. Learn more about caring for a terminally ill child and advanced care planning.

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving. Learn more about grieving the loss of a child.

Some families find comfort in getting involved in research efforts to advance knowledge about brain stem gliomas. Learn more about tissue donation. (Please note that this link takes you to a separate website.)