Brain Stem Glioma - Childhood

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What is Brain Stem Glioma - Childhood?

About the brain stem

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

About brain stem glioma

Brain stem glioma is a type of central nervous system (CNS; brain and spinal cord) tumor that begins when normal cells in the brain stem change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). A glioma is a tumor that grows from a glial cell, which is a supportive cell in the brain.

Brain stem glioma is most often diffuse (spread freely) through the brain stem by the time of diagnosis. 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 a low-grade tumor (the tumor cells look similar to normal cells) that is less likely to grow and spread quickly.

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 .

Symptoms and Signs

Children with a brain stem glioma may experience the following symptoms or signs. Sometimes, children with a 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 or sign on this list, please talk with your child's doctor.

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

Your child's doctor will ask you questions about the symptoms your child is experiencing to help find out the cause of the problem, called a diagnosis. This may include how long your child has been experiencing the symptom(s) and how often.

If a tumor is diagnosed, relieving symptoms and side effects remains an important part of care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with the health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

Risk Factors

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 risk factors never develop a tumor, while others with no known risk factors do.

Doctors and researchers don't 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. 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.

Diagnosis

Doctors use many tests to diagnose a brain stem glioma and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most other 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 it can have major risks. In addition, the diagnosis can often be made by magnetic resonance imaging (MRI) alone (see below). Because of this, diffuse brain stem glioma is unlike most other tumors. For a focal tumor, 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. Other imaging tests may be used to find out whether the tumor has metastasized. Your child's doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of tumor suspected

Severity of symptoms

Previous test results

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

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium (a special dye) may be injected into a patient's vein to create a clearer picture.

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 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 still needed.

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 find out the type of tumor. If possible, a neurosurgeon (a doctor who specializes in treating a CNS tumor using surgery) will remove a small piece of tissue from the brain. The sample removed during the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).

Stages

Staging is a way of describing where the tumor is located, if or where it has spread, and whether 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 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 describes how much the tumor cells look like normal cells. A low-grade tumor is less aggressive than a high-grade tumor.

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 in one 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 fairly normal.

Recurrent brain stem glioma: Recurrent brain stem glioma is a tumor that comes back after treatment. If there is a recurrence, the tumor may need to be graded again using the system above.

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