Childhood Brain Stem Glioma

Key Points for This Section

* Childhood brain stem glioma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain stem.
* The cause of most childhood brain tumours is unknown.
* The symptoms of childhood brain stem glioma vary and often depend on the child’s age and where the tumour is located.
* Tests that examine the brain are used to detect (find) childhood brain stem glioma.
* Childhood brain stem glioma is diagnosed and removed in surgery.
* Certain factors affect prognosis (chance of recovery) and treatment options.

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.

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

This summary refers to the treatment of primary brain tumours (tumours that begin in the brain). Treatment for metastatic brain tumours, which are tumours formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary. Brain tumours can occur in both children and adults; however, treatment for children may be different than treatment for adults. (Refer to the treatment summary on Adult Brain Tumours for more information.)

The cause of most childhood brain tumours is unknown.

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

These symptoms may be caused by a brain stem glioma or other conditions. A doctor should be notified if any of the following problems occur:

* Loss of balance and difficulty 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, computerised tomography, or computerised 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 the patient through 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 tumour 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 tumour 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 it 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 tumour remaining. The extent or spread of cancer is usually described as stages. For childhood brain stem glioma, the tumours are described by type:

* Diffuse intrinsic glioma is a tumour that has spread widely throughout the brain stem.
* Focal or low-grade glioma is a tumour that is localised to one area of the brain stem.

Recurrent Childhood Brain Stem Glioma

Recurrent childhood brain stem glioma is a tumour 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 tumour may come back in the brain or in other areas of the central nervous system.

Treatment Option Overview

Key Points for This Section

* There are different types of treatment for children with brain stem glioma.
* Children with brain stem glioma should have their treatment planned by a team of doctors with expertise in treating childhood brain tumours.
* Four types of standard treatment are used:
o Surgery
o Radiation therapy
o Chemotherapy
o Cerebrospinal fluid diversion
* Other types of treatment are being tested in clinical trials.
o Radiation therapy with radiosensitizers

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. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Cancer.gov Web site. 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 tumours.

Your child’s treatment will be overseen by a paediatric oncologist, a doctor who specializes in treating children with cancer. The paediatric oncologist may refer you to other paediatric doctors who have experience and expertise in treating children with brain tumours and who specialize in certain areas of medicine. These may include the following specialists:

* Neurosurgeon.
* Neurologist.
* Neuropathologist.
* Neuroradiologist.
* Rehabilitation specialist.
* Radiation oncologist.
* Medical oncologist.
* Endocrinologist.
* Psychologist.

Four 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. 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, a body cavity such as the abdomen, or an organ, the drugs mainly affect cancer cells in those areas. 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.

Other types of treatment are being tested in clinical trials.

Radiation therapy with radiosensitizers

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

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Cancer.gov Web site.

Treatment Options for Childhood Brain Stem Glioma

Untreated Childhood Brain Stem Glioma

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

Standard treatment of diffuse intrinsic brain stem glioma may include the following:

* Radiation therapy.

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

* A clinical trial of chemotherapy combined with radiation therapy.
* A clinical trial of chemotherapy to delay the use of radiation therapy.

Information about these and other ongoing clinical trials is available from the NCI Cancer.gov Web site

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

* Surgery.
* Cerebrospinal fluid diversion followed by careful observation (also called watchful waiting). Watchful waiting is closely monitoring a patient’s condition but withholding treatment until symptoms appear or change.

Some of the treatments being studied in clinical trials for focal or low-grade glioma include the following:

* A clinical trial of surgery followed by watchful waiting.

Information about this and other ongoing clinical trials is available from the NCI Cancer.gov Web site

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

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

Standard treatment of recurrent diffuse intrinsic brain stem glioma may include the following:

* Surgery.
* Palliative therapy to relieve symptoms and improve quality of life.

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

* A clinical trial of a new therapy.

Information about this and other ongoing clinical trials is available from the NCI Cancer.gov Web site.

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

* Surgery.
* Chemotherapy.