Childhood Medulloblastoma

Key Points for This Section

* Childhood medulloblastoma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain.
* The cause of most childhood brain tumours is unknown.
* The signs of childhood medulloblastoma 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 medulloblastoma.
* Childhood medulloblastoma is diagnosed and removed in surgery.
* Certain factors affect prognosis (chance of recovery) and treatment options.

Childhood medulloblastoma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain.

Childhood medulloblastoma (tumour) usually forms in the cerebellum, which is at the lower back of the brain. The cerebellum is the part of the brain that controls movement, balance, and posture. Childhood medulloblastoma may also be called primitive neuroectodermal tumour (PNET).

About 1 out of 5 childhood brain tumours are medulloblastomas. 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 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 signs of childhood medulloblastoma vary and often depend on the child’s age and where the tumour is located.

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

* Loss of balance, difficulty walking, worsening handwriting, or slow speech.
* Morning headache or headache that goes away after vomiting.
* Nausea and vomiting.
* Unusual sleepiness or change in energy level.
* Change in personality or behaviour.
* Unexplained weight loss or weight gain.

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

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 medulloblastoma 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 tumour 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 possible during the same surgery.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on:

* The age of the child when the tumour is found.
* The location of the tumour.
* The amount of tumour remaining after surgery.
* Whether the cancer has spread to other parts of the central nervous system (brain and spinal cord), or to other parts of the body, such as the bones.

Stages of Childhood Medulloblastoma

Key Points for This Section

* After childhood medulloblastoma has been diagnosed, tests are done to find out if there is tumour remaining or if cancer cells have spread.
* The following risk categories are used for childhood medulloblastoma:
o Average risk
o Poor risk

After childhood medulloblastoma has been diagnosed, tests are done to find out if there is tumour remaining or if cancer cells have spread.

The extent or spread of cancer is usually described as stages. For childhood medulloblastoma, risk groups are used instead of stages. Risk groups are described by the amount of remaining tumour or spread of cancer cells within the central nervous system (brain and spinal cord) or to other parts of the body. It is important to know the risk group in order to plan treatment. The following tests and procedures may be used to determine the risk group:

* 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.
* Bone marrow biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and the bone marrow samples under a microscope to look for signs of cancer.
* Bone scan: A procedure to detect if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the blood stream. The radioactive material collects in the bones and is detected by a scanner.

The following risk categories are used for childhood medulloblastoma:

Average risk

Childhood medulloblastoma is called an average risk if all of the following are true:

* The child is older than 3 years of age.
* The tumour is at the very back of the brain.
* All of the tumour was removed by surgery or there was a very small amount remaining.
* The cancer has not spread to other parts of the body.

Poor risk

Childhood medulloblastoma is called a poor risk if any of the following are true:

* The child is younger than 3 years of age.
* The tumour is not at the very back of the brain.
* Some of the tumour was not removed by surgery.
* The cancer has spread to other parts of the body.

In general, cancer is more likely to recur (come back) in patients in the poor risk group.

Recurrent Childhood Medulloblastoma

Recurrent childhood medulloblastoma is a tumour that has recurred (come back) after it has been treated. Childhood medulloblastoma often recurs. A tumour may come back many years later at the same place or a different place in the brain. It can also come back in other parts of the body such as the spinal cord.

Treatment Option Overview

Key Points for This Section

* There are different types of treatment for children with childhood medulloblastoma.
* Children with medulloblastoma 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 High-dose chemotherapy with bone marrow transplantation or stem cell transplantation and radiation therapy

There are different types of treatment for children with childhood medulloblastoma.

Different types of treatment are available for children with childhood medulloblastoma. 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 medulloblastoma 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 medulloblastoma 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.

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.

Because radiation therapy 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.

High-dose chemotherapy with bone marrow transplantation or stem cell transplantation and radiation therapy

High-dose chemotherapy with bone marrow or stem cell transplantation is a method of giving high doses of chemotherapy and then replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells. Clinical trials are studying the use of this treatment followed by radiation therapy directed at the tumour only for childhood medulloblastoma.

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 Medulloblastoma

Untreated Childhood Medulloblastoma

Untreated childhood medulloblastoma 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 average risk childhood medulloblastoma may include the following:

* Surgery.
* Radiation therapy to the brain and spinal cord with or without chemotherapy.
* A clinical trial of new combinations of radiation therapy and chemotherapy for children 3 years and older.

Standard treatment of poor risk childhood medulloblastoma may include the following:

* Surgery.
* Radiation therapy to the brain and spinal cord.
* Chemotherapy.

Standard treatment of childhood medulloblastoma in children younger than 3 years of age may include the following:

* Surgery with or without chemotherapy.
* Chemotherapy.
* Radiation therapy with or without chemotherapy.

Some of the treatments being studied in clinical trials for untreated childhood medulloblastoma in children younger than 3 years of age include the following:

* A clinical trial of chemotherapy to delay or reduce the need for radiation therapy.
* A clinical trial of high-dose chemotherapy with bone marrow transplantation followed by radiation therapy.
* A clinical trial of chemotherapy followed by surgery and radiation therapy.

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

Standard treatment of recurrent childhood medulloblastoma may include the following:

* Surgery with or without chemotherapy.

Some of the treatments being studied in clinical trials for recurrent childhood medulloblastoma include the following:

* A clinical trial of high-dose chemotherapy and bone marrow transplantation or peripheral blood stem cell transplantation with radiation therapy.
* A clinical trial of a new therapy.