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 pontine gliomas, which form in a part of the brain stem called the pons.

Although cancer is rare in children, brain tumors are the most common type of childhood cancer other than leukemia or lymphoma.

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.

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.

These and other symptoms may be caused by a brain stem glioma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following 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 tests and procedures 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): 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).

Childhood brain stem glioma is diagnosed and removed in surgery.

If the tumor has not spread widely within the brain stem, 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) depends on:

  • The type of brain stem glioma.
  • Where the tumor is located and if it has spread within the brain stem.
  • Whether or not the child has an underlying condition called neurofibromatosis type 1.
  • Whether the glioma has just been diagnosed or has recurred (come back).

Treatment options depend on the type and location of the glioma.

Stages of Childhood Brain Stem Glioma

After the childhood brain stem glioma has been removed, tests are done to find out if there is tumor remaining. The extent or spread of cancer is usually described as stages. For childhood brain stem glioma, the tumors are described by type:

  • Diffuse intrinsic pontine glioma is a tumor that has spread widely throughout the brain stem.
  • Focal or low-grade glioma is a tumor that is localized to one area of the brain stem.

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.  Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Children with brain stem glioma should have their treatment planned by a team of doctors with expertise in treating childhood brain tumors.

Your child’s treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist may refer you to other pediatric doctors who have experience and expertise 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.

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. 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 uses a computer to create a 3-D picture of the tumor. The radiation beams are then shaped to fit the tumor.
  • Hyperfractionated radiation therapy is a way of giving radiation therapy in smaller-than-usual doses two or three times a day instead of 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.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells 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 spinal column, 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 (hollow 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.

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.

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.

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 pontine glioma is usually radiation therapy.

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

  • A clinical trial of chemotherapy combined with radiation therapy.
  • A clinical trial of a new kind of treatment given during and/or after radiation therapy.

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

  • Surgery with or without radiation therapy.
  • 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.

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 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 pontine glioma is usually palliative therapy, to relieve symptoms and improve the patient's quality of life. Standard treatment of recurrent focal or low-grade childhood brain stem glioma may include the following:

  • Surgery.
  • Radiation therapy.
  • Chemotherapy.

Source:  National Cancer Institute