Childhood Ependymoma

Key Points for This Section

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

Childhood ependymoma is a disease in which malignant (cancer) cells form in the tissues of the brain and spinal cord.

The brain controls vital functions such as memory and learning, the senses (hearing, sight, smell, taste, and touch), and emotion. The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body.

About 1 in 11 childhood brain tumours are ependymomas. Although cancer is rare in children, brain tumours are the most common type of childhood cancer other than leukaemia and lymphoma.

This summary refers to the treatment of primary brain tumours (tumours that begin in the brain). Treatment of 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.

There are many different types of brain tumours. Brain tumours can occur in both children and adults; however, treatment for children may be different than treatment for adults. Refer to the following summaries for more information:

* Childhood Brain Tumours Treatment
* Adult Brain Tumours Treatment

The cause of most childhood brain tumours is unknown.

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

These symptoms may be caused by childhood ependymoma or other conditions. A doctor should be consulted if any of the following problems occur:

* Frequent headaches.
* Seizures.
* Frequent nausea and vomiting.
* Loss of balance or trouble walking.

Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma.

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 ependymoma is diagnosed and removed in surgery.

If a brain tumour is suspected, a biopsy is 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).

The prognosis (chance of recovery) depends on:

* Amount of tumour removed during surgery.
* Tumour histology (how the tumour cells look under a microscope).
* The age of the child when the tumour was found.

Stages of Childhood Ependymoma

After the childhood ependymoma 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 ependymoma, tumours are described by grade and by where they are located in the central nervous system (brain and spinal cord). The grade of the tumour refers to how abnormal the cancer cells look under a microscope and how quickly the tumour is likely to grow and spread. It is important to know the grade of the tumour and if there were any cancer cells remaining after surgery in order to plan treatment. The following tests and procedures may be used:

* 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).
* Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.

Recurrent Childhood Ependymoma

Recurrent childhood ependymoma is a tumour that has recurred (come back) after it has been treated. Childhood ependymoma commonly recurs, usually at the original cancer site. The tumour may come back as long as 15 years or more after initial treatment.

Treatment Option Overview

Key Points for This Section

* There are different types of treatment for children with ependymoma.
* Children with ependymoma should have their treatment planned by a team of doctors with expertise in treating childhood brain tumours.
* Three types of standard treatment are used:
o Surgery
o Radiation therapy
o Chemotherapy
* New types of treatment are being tested in clinical trials.

There are different types of treatment for children with ependymoma.

Different types of treatment are available for children with ependymoma. 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 Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

Children with ependymoma 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.

Some cancer treatments cause side effects that continue or appear years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include physical problems; changes in mood, feelings, thinking, learning or memory; and having second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. Refer to the summary on Late Effects of Treatment for Childhood Cancer for more information.

Three types of standard treatment are used:

Surgery

Surgery is used to diagnose and treat childhood ependymoma as described 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. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Certain ways of giving radiation therapy can help keep radiation away from healthy tissue:

* Conformal radiation therapy uses a computer to create a 3-D picture of the tumour. The radiation beams are shaped to fit the tumour.
* Stereotactic radiation therapy uses a head frame attached to the skull to aim radiation beams directly at the tumour.

Radiation therapy to the brain can affect growth and development in young children and is not standard treatment for children younger than 3 years.

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 into 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.

New types of treatment are being tested in clinical trials.

Information about ongoing clinical trials is available from the NCI Web site.

Treatment Options for Childhood Ependymoma

Newly Diagnosed Childhood Ependymoma

Newly diagnosed childhood ependymoma is a tumour that has not been treated. The patient may have received drugs or treatment, however, to relieve symptoms caused by the tumour.

Initial treatment for newly diagnosed childhood ependymoma is usually surgery, with or without additional treatment.

After surgery, treatment depends on the age of the child, the amount of tumour that was removed, and whether cancer cells have spread to other parts of the central nervous system.

When the tumour is completely removed by surgery and cancer cells have not spread within the central nervous system, treatment may include the following:

* Radiation therapy to the tumour bed (where the tumour was before it was removed), for children aged 3 years or older.
* A clinical trial of watchful waiting, for children aged 1 year or older.
* A clinical trial of conformal radiation therapy.
* A clinical trial of chemotherapy with or without radiation therapy.

When a part of the tumour remains after surgery, but cancer cells have not spread within the central nervous system, treatment may include the following:

* Second-look surgery.
* Radiation therapy to the tumour bed, for children aged 3 years or older.
* A clinical trial of chemotherapy followed by second-look surgery and conformal radiation therapy, for children aged 1 year or older.

When cancer cells have spread within the central nervous system, treatment may include the following:

* Radiation therapy to the whole brain and spine.
* A clinical trial of radiation therapy and chemotherapy.

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

Standard treatment of recurrent childhood ependymomas may include the following:

* Surgery.
* Radiation therapy, including stereotactic radiation therapy with or without chemotherapy.
* Chemotherapy with or without radiation.