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Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®)

Patient Version
Last Modified: 02/08/2012

General Information About Childhood Central Nervous System Embryonal Tumors

Key Points for This Section


Central nervous system embryonal tumors begin in embryonic (fetal) cells in the brain and spinal cord.

Central nervous system (CNS) embryonal tumors form in brain cells when the fetus is beginning to develop.

The tumors may be benign (not cancer) or malignant (cancer). Most CNS embryonal tumors in children are malignant. Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause symptoms and need treatment.

Although cancer is rare in children, brain tumors are the third most common type of childhood cancer, after leukemia and lymphoma. This summary discusses the treatment of primary brain tumors (tumors that begin in the brain). The treatment of metastatic brain tumors, which 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. See the PDQ treatment summary on Adult Brain Tumors 1 for more information.

CNS embryonal tumors may form in different areas of the brain.

Childhood brain tumors are named based on the type of cell they formed in and where the tumor first formed in the brain.

Enlarge
Drawing of the inside of the brain showing ventricles (fluid-filled spaces), choroid plexus, hypothalamus, pineal gland, pituitary gland, optic nerve, brain stem, cerebellum, cerebrum, medulla, pons, and spinal cord.
Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain.

There are 6 different types of CNS embryonal tumors:

Medulloblastoma

Medulloblastomas are fast-growing tumors that form in brain cells in the cerebellum. The cerebellum is at the lower back part of the brain between the cerebrum and the brain stem. The cerebellum controls movement, balance, and posture. Sometimes medulloblastoma spreads to the bone, bone marrow, lymph nodes, liver, or lung.

Pineal gland tumors

The pineal gland is a tiny organ in the center of the brain. The gland makes melatonin, a substance that helps control our sleep cycle. Childhood tumors of the pineal gland include the following:

  • Pineoblastomas: Fast-growing tumors that form in cells of the pineal gland. The tumor cells look very different from normal pineal gland cells. Pineoblastomas are usually malignant.
  • Pineal parenchymal tumors of intermediate differentiation: Slow-growing or fast-growing tumors that form in the pineal gland. Some of the tumor cells almost look like normal pineal gland cells and other tumor cells look very different from normal pineal gland cells.

Central nervous system primitive neuroectodermal tumors

Central nervous system primitive neuroectodermal tumors are fast-growing tumors that form in brain cells in the cerebrum. The cerebrum is at the top of the head and is the largest part of the brain. The cerebrum controls thinking, learning, problem-solving, emotions, speech, reading, writing, and voluntary movement.

Medulloepithelioma

Medulloepitheliomas are fast-growing tumors that form in brain cells that line tubelike spaces in the brain and spinal cord. These rare tumors are most common in infants and young children.

Ependymoblastoma

Ependymoblastomas are fast-growing tumors that form in brain cells lining the fluid -filled spaces in the brain and spinal cord, where it is often near the tailbone. These rare tumors are most common in infants and young children.

Childhood CNS atypical teratoid/rhabdoid tumor is also a type of embryonal tumor, but it is treated differently than other childhood CNS embryonal tumors. See the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment 2 for more information.

Certain genetic conditions increase the risk of childhood CNS embryonal tumors.

Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Parents who think their child may be at risk should discuss this with the child's doctor. Risk factors for CNS embryonal tumors include having the following inherited diseases:

In most cases, the cause of CNS embryonal tumors is not known.

The symptoms of childhood CNS embryonal tumors are not the same in every child.

The following symptoms and others may be caused by a CNS embryonal tumor. Symptoms vary depending on the child's age and where the tumor is located. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Loss of balance, trouble walking, worsening handwriting, or slow speech.
  • Morning headache or headache that goes away after vomiting.
  • Nausea and vomiting.
  • General weakness or weakness on one side of the face.
  • Unusual sleepiness or change in energy level.
  • Change in personality or behavior.
  • Unexplained weight loss or weight gain.

An infant or young child may be irritable or grow slowly, and may not eat well or meet developmental milestones such as sitting, walking, and talking in sentences.

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

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a patient's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
  • MRI (magnetic resonance imaging) of the brain and spinal cord with gadolinium. 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). Sometimes magnetic resonance spectroscopy (MRS) is done during the same MRI scan to look at the chemical makeup of the brain tissue.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of the brain and spinal cord, 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.
  • 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.
  • SPECT scan (single photon emission computed tomography): A procedure that uses a special camera linked to a computer to make a 3-dimensional (3-D) picture of the brain. A small amount of a radioactive substance is injected into a vein or inhaled through the nose. As the substance travels through the blood, the camera rotates around the head and takes pictures of the brain. There will be increased blood flow and more chemical reactions (metabolism) in areas where cancer cells are growing. These areas will show up brighter in the picture. This procedure may be done just before or after a CT scan.
  • PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the brain. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

Childhood CNS embryonal tumors are usually diagnosed and removed in surgery.

If doctors think your child may have a CNS embryonal tumor, a biopsy may be done to remove a sample of tissue. For brain tumors, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. Sometimes, a computer-guided needle is used to remove a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery.

The following tests may be done on the sample of tissue that is removed:

  • Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
  • Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
  • Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.

Some pineoblastoma and pineal parenchymal tumors are diagnosed by brain imaging tests.

Sometimes, it is not possible to do a safe biopsy or completely remove the tumor in surgery, because of where it is in the brain. These tumors are diagnosed based on the results of imaging tests and other procedures.

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 tumor is found.
  • The type of tumor and where it is in the brain.
  • Whether the cancer has spread within the brain and spinal cord or to other parts of the body, such as the bones.
  • Whether there are certain changes in the chromosomes or genes.
  • Whether the tumor has just been diagnosed or has recurred (come back).

Staging Childhood Central Nervous System Embryonal Tumors

Key Points for This Section


Treatment is based on the child’s risk group.

Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment.

There is no standard staging system for childhood central nervous system (CNS) embryonal tumors. Instead, treatment depends on the child’s risk group:

Average risk

Childhood CNS embryonal tumors are called average risk when all of the following are true:

  • The child is older than 3 years of age.
  • All of the tumor was removed by surgery or there was only a very small amount remaining.
  • The cancer has not spread to other parts of the body.

High risk

Childhood CNS embryonal tumors are called high risk if any of the following are true:

  • The child is 3 years of age or younger.
  • Some of the tumor 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 high-risk group.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

The information from tests and procedures done to detect (find) childhood CNS embryonal tumors is used to plan cancer treatment.

Some of the tests used to detect childhood CNS embryonal tumors are repeated after the tumor is removed by surgery. (See the General Information 3 section.) This is to find out how much tumor remains after surgery. Other tests and procedures may be done to find out if the cancer has spread:

Recurrent Childhood Central Nervous System Embryonal Tumors

A recurrent childhood central nervous system (CNS) embryonal tumor is a tumor that recurs (comes back) after being treated. Childhood CNS embryonal tumors most often recur within 18 months after treatment but may come back many years later. Recurrent childhood CNS embryonal tumors may come back in the same place as the original tumor or in a different place in the brain or spinal cord.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood brain tumor 4. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 5.

Treatment Option Overview

Key Points for This Section


There are different types of treatment for children who have CNS embryonal tumors.

Different types of treatment are available for children with central nervous system (CNS) embryonal tumors. 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. Some clinical trials are open only to patients who have not started treatment.

Children who have CNS embryonal tumors should have their treatment planned by a team of health care providers who are experts in treating brain tumors in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:

Childhood brain tumors may cause symptoms that begin before diagnosis and continue for months or years.

Symptoms caused by the tumor may begin before diagnosis. These symptoms may continue for months or years. It is important to talk with your child's doctors about symptoms caused by the tumor that may continue after treatment.

Some cancer treatments cause side effects months or years after treatment has ended.

Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).

Children diagnosed with medulloblastoma may have certain problems after surgery or radiation therapy such as changes in the ability to think, learn, and pay attention. Also, cerebellar mutism syndrome may occur after surgery. Symptoms of this syndrome include the following:

  • Not being able to speak.
  • Trouble swallowing and eating.
  • Loss of balance, trouble walking, and worsening handwriting.
  • Loss of muscle tone.
  • Mood swings and changes in personality.

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer 6 for more information).

Three types of standard treatment are used:

Surgery

Surgery is used to diagnose and treat a childhood CNS embryonal tumor as described in the General Information 7 section of this summary.

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy and/or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. 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. For this reason, clinical trials are studying new ways of giving radiation that may have fewer side effects than standard methods. For childhood CNS embryonal tumors, radiation therapy may be given in the following ways:

  • Conformal radiation therapy uses a computer to make a 3-dimensional (3-D) picture of the tumor and the radiation beams are shaped to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to normal tissue around the tumor.
  • Stereotactic radiation therapy uses a rigid head frame attached to the skull to aim radiation directly to a tumor, causing less damage to normal tissue around the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.

The way the radiation therapy is given depends on the type of cancer being treated.

Because radiation therapy can affect growth and brain development in young children, especially children who are three years old or younger, chemotherapy may be given to delay or reduce the need for radiation therapy.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them 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 cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type of cancer being treated.

Anticancer drugs given by mouth or vein to treat central nervous system tumors cannot cross the blood-brain barrier and enter the fluid that surrounds the brain and spinal cord. Instead, an anticancer drug is injected into the fluid-filled space to kill cancer cells that may have spread there. This is called intrathecal chemotherapy.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site 8.

High-dose chemotherapy with stem cell rescue

High-dose chemotherapy with stem cell rescue is a way of giving high doses of chemotherapy and 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.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. (See the General Information 3 section for a list of tests.) Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the brain tumor has recurred (come back). If the tumor recurs in the brain, a biopsy may also be done to find out if it is made up of dead tumor cells or if new cancer cells are growing. These tests are sometimes called follow-up tests or check-ups.

Treatment Options for Childhood Central Nervous System Embryonal Tumors

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Newly Diagnosed Childhood Medulloblastoma

In newly diagnosed childhood medulloblastoma, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Average risk

Standard treatment of average-risk childhood medulloblastoma is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes combination chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Clinical trials are studying new combinations of radiation therapy, including conformal radiation therapy, and chemotherapy.

High risk

Standard treatment of high-risk childhood medulloblastoma is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes combination chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Clinical trials are studying new combinations of radiation therapy and chemotherapy.

Children 3 years old or younger

Standard treatment of childhood medulloblastoma in children 3 years old or younger is usually surgery followed by chemotherapy. Other treatments may include the following:

  • Surgery followed by combination chemotherapy.
  • Surgery followed by high-dose chemotherapy with stem cell rescue.
  • Surgery followed by combination chemotherapy and radiation therapy to the area where the tumor was removed.

Treatment of medulloblastoma in children 3 years old or younger is often within a clinical trial. Clinical trials are studying new combinations and schedules of chemotherapy with stem cell rescue.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated childhood medulloblastoma 9. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 5.

Newly Diagnosed Childhood Pineoblastoma and Pineal Parenchymal Tumors

In newly diagnosed childhood pineoblastoma or pineal parenchymal tumors, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Children older than 3 years

Standard treatment of childhood pineoblastoma and pineal parenchymal tumors in children older than 3 years is usually surgery. It is usually not possible to remove all of the tumor, because of where it is in the brain. Surgery is usually followed by radiation therapy to the brain and spinal cord. Sometimes combination chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Clinical trials are studying new treatments for high-risk childhood pineoblastoma, including combinations of chemotherapy and radiation therapy.

Children 3 years old or younger

Treatment of pineoblastoma and pineal parenchymal tumors in children 3 years old or younger may include surgery followed by chemotherapy. Radiation therapy may be given when the child is older. High-dose chemotherapy with stem cell rescue has been used.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated childhood pineoblastoma 10 and childhood pineal parenchymal tumor 11. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 5.

Newly Diagnosed Childhood Central Nervous System Primitive Neuroectodermal Tumors

In newly diagnosed childhood central nervous system (CNS) primitive neuroectodermal tumors, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Children older than 3 years

Standard treatment of CNS primitive neuroectodermal tumors in children older than 3 years is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes combination chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Children 3 years old or younger

Standard treatment of CNS primitive neuroectodermal tumors in children 3 years old or younger is usually surgery followed by chemotherapy. Other treatments may include the following:

  • Surgery followed by combination chemotherapy.
  • Surgery followed by high-dose chemotherapy with stem cell rescue.
  • Surgery followed by combination chemotherapy and radiation therapy to the area where the tumor was removed.

Treatment of CNS primitive neuroectodermal tumors in children 3 years old or younger is often within a clinical trial. Clinical trials are studying new combinations of chemotherapy with stem cell rescue.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated childhood supratentorial primitive neuroectodermal tumor 12. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 5.

Newly Diagnosed Childhood Medulloepithelioma and Ependymoblastoma

In newly diagnosed childhood medulloepithelioma and ependymoblastoma, the tumor itself has not been treated. The child may have received drugs or treatment to relieve symptoms caused by the tumor.

Children older than 3 years

Standard treatment of childhood medulloepithelioma or ependymoblastoma in children older than 3 years is usually surgery followed by radiation therapy to the brain and spinal cord. Sometimes combination chemotherapy is given at the same time as radiation therapy or after radiation therapy.

Children 3 years old or younger

Standard treatment of childhood medulloepithelioma or ependymoblastoma in children 3 years old or younger is usually surgery followed by chemotherapy. Other treatments may include the following:

  • Surgery followed by combination chemotherapy.
  • Surgery followed by high-dose chemotherapy with stem cell rescue.
  • Surgery followed by combination chemotherapy and radiation therapy to the area where the tumor was removed.

Treatment of childhood medulloepithelioma or ependymoblastoma in children 3 years old or younger is often within a clinical trial.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood ependymoblastoma 13 and childhood medulloepithelioma 14. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 5.

Recurrent Childhood Central Nervous System Embryonal Tumors

Treatment of recurrent childhood CNS embryonal tumors may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood central nervous system embryonal tumor 15. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 5.

To Learn More About Childhood Central Nervous System Embryonal Tumors

For more information from the National Cancer Institute about childhood central nervous system embryonal tumor, see the following:

For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:

Get More Information From NCI

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There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

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Changes to This Summary (02/08/2012)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site 30. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site 5. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site 30 or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).



Glossary Terms

3-dimensional (3-dih-MEN-shuh-nul)
A graphic display of depth, width, and height. Also called 3-D.
antibody (AN-tee-BAH-dee)
A protein made by plasma cells (a type of white blood cell) in response to an antigen (a substance that causes the body to make a specific immune response). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.
antigen (AN-tih-jen)
Any substance that causes the body to make a specific immune response.
atypical teratoid/rhabdoid tumor (AY-TIH-pih-kul TAYR-uh-toyd/RAB-doyd TOO-mer)
An aggressive cancer of the central nervous system, kidney, or liver that occurs in very young children. Also called AT/RT and ATT/RHT.
benign (beh-NINE)
Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.
biopsy (BY-op-see)
The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
blood (blud)
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
bone marrow (bone MAYR-oh)
The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.
brain stem (brayn stem)
The part of the brain that is connected to the spinal cord.
brain tumor (brayn TOO-mer)
The growth of abnormal cells in the tissues of the brain. Brain tumors can be benign (not cancer) or malignant (cancer).
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
central nervous system (SEN-trul NER-vus SIS-tem)
The brain and spinal cord. Also called CNS.
cerebellum (SAYR-eh-BEH-lum)
The portion of the brain in the back of the head between the cerebrum and the brain stem. The cerebellum controls balance for walking and standing, and other complex motor functions.
cerebrospinal fluid (seh-REE-broh-SPY-nul FLOO-id)
The fluid that flows in and around the hollow spaces of the brain and spinal cord, and between two of the meninges (the thin layers of tissue that cover and protect the brain and spinal cord). Cerebrospinal fluid is made by tissue called the choroid plexus in the ventricles (hollow spaces) in the brain. Also called CSF.
cerebrum (seh-REE-brum)
The largest part of the brain. It is divided into two hemispheres, or halves, called the cerebral hemispheres. Areas within the cerebrum control muscle functions and also control speech, thought, emotions, reading, writing, and learning.
chromosome (KROH-muh-some)
Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.
clinical trial (KLIH-nih-kul TRY-ul)
A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.
condition (kun-DIH-shun)
In medicine, a health problem with certain characteristics or symptoms.
contrast material (KON-trast muh-TEER-ee-ul)
A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.
CT scan (… skan)
A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
cytogenetics (SY-toh-jeh-NEH-tix)
The study of chromosomes and chromosomal abnormalities.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
differentiation (DIH-feh-REN-shee-AY-shun)
In cancer, refers to how mature (developed) the cancer cells are in a tumor. Differentiated tumor cells resemble normal cells and tend to grow and spread at a slower rate than undifferentiated or poorly differentiated tumor cells, which lack the structure and function of normal cells and grow uncontrollably.
electron microscope (ee-LEK-tron MY-kroh-SKOPE)
A microscope (device used to magnify small objects) that uses electrons (instead of light) to produce an enlarged image. An electron microscope shows tiny details better than any other type of microscope.
embryonal tumor (em-BRY-uh-nul TOO-mer)
A mass of rapidly growing cells that begins in embryonic (fetal) tissue. Embryonal tumors may be benign or malignant, and include neuroblastomas and Wilms tumors. Also called embryoma.
fetus (FEE-tus)
In humans, an unborn baby that develops and grows inside the uterus (womb). The fetal period begins 8 weeks after fertilization of an egg by a sperm and ends at the time of birth.
fluid (FLOO-id)
A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.
gadolinium (GA-duh-LIH-nee-um)
A metal element that is used in magnetic resonance imaging (MRI) and other imaging methods. It is a contrast agent, which helps show abnormal tissue in the body during imaging with a special machine.
gene (jeen)
The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.
glucose (GLOO-kose)
A type of sugar; the chief source of energy for living organisms.
imaging test (IH-muh-jing …)
A type of test that makes pictures of areas inside the body. Some examples of imaging tests are CT scans and MRIs. Also called imaging procedure.
immunohistochemistry (IH-myoo-noh-HIS-toh-KEH-mih-stree)
A technique used to identify specific molecules in different kinds of tissue. The tissue is treated with antibodies that bind the specific molecule. These are made visible under a microscope by using a color reaction, a radioisotope, colloidal gold, or a fluorescent dye. Immunohistochemistry is used to help diagnose diseases, such as cancer, and to detect the presence of microorganisms. It is also used in basic research to understand how cells grow and differentiate (become more specialized).
inhalation (IN-huh-LAY-shun)
In medicine, refers to the act of taking a substance into the body by breathing.
inherited (in-HAYR-ih-ted)
Transmitted through genes that have been passed from parents to their offspring (children).
injection (in-JEK-shun)
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
laboratory test (LA-bruh-tor-ee...)
A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.
leukemia (loo-KEE-mee-uh)
Cancer that starts in blood-forming tissue such as the bone marrow and causes large numbers of blood cells to be produced and enter the bloodstream.
Li-Fraumeni syndrome (lee-FRAH-meh-nee SIN-drome)
A rare, inherited predisposition to multiple cancers, caused by an alteration in the p53 tumor suppressor gene.
liver (LIH-ver)
A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.
lumbar puncture (LUM-bar PUNK-cher)
A procedure in which a thin needle called a spinal needle is put into the lower part of the spinal column to collect cerebrospinal fluid or to give drugs. Also called spinal tap.
lung (lung)
One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.
lymph node (limf node)
A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.
lymphoma (lim-FOH-muh)
Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.
malignant (muh-LIG-nunt)
Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.
medical history (MEH-dih-kul HIH-stuh-ree)
A record of information about a person’s health. A personal medical history may include information about allergies, illnesses, surgeries, immunizations, and results of physical exams and tests. It may also include information about medicines taken and health habits, such as diet and exercise. A family medical history includes health information about a person’s close family members (parents, grandparents, children, brothers, and sisters). This includes their current and past illnesses. A family medical history may show a pattern of certain diseases in a family.
medulloblastoma (MED-yoo-loh-blas-TOH-muh)
A malignant brain tumor that begins in the lower part of the brain and that can spread to the spine or to other parts of the body. Medulloblastomas are a type of primitive neuroectodermal tumor (PNET).
melatonin (MEH-luh-TOH-nin)
A hormone made by the pineal gland (tiny organ near the center of the brain). Melatonin helps control the body’s sleep cycle, and is an antioxidant. It is also made in the laboratory and sold as a supplement.
metabolism (meh-TA-buh-lih-zum)
The chemical changes that take place in a cell or an organism. These changes make energy and the materials cells and organisms need to grow, reproduce, and stay healthy. Metabolism also helps get rid of toxic substances.
metastatic (meh-tuh-STA-tik)
Having to do with metastasis, which is the spread of cancer from the primary site (place where it started) to other places in the body.
microscope (MY-kroh-SKOPE)
An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.
MRI
A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.
nausea (NAW-zee-uh)
A feeling of sickness or discomfort in the stomach that may come with an urge to vomit. Nausea is a side effect of some types of cancer therapy.
nerve (nerv)
A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.
neurological exam (NOOR-oh-LAH-jih-kul eg-ZAM)
A series of questions and tests to check brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, ability to walk, and how well the muscles, sensory systems, and deep tendon reflexes work.
nevoid basal cell carcinoma syndrome (NEE-voyd BAY-sul sel KAR-sih-NOH-muh SIN-drome)
A genetic condition that causes unusual facial features and disorders of the skin, bones, nervous system, eyes, and endocrine glands. People with this syndrome have a higher risk of basal cell carcinoma. Also called basal cell nevus syndrome and Gorlin syndrome.
organ (OR-gun)
A part of the body that performs a specific function. For example, the heart is an organ.
parenchyma (puh-REN-kih-muh)
The essential or functional elements of an organ.
pathologist (puh-THAH-loh-jist)
A doctor who identifies diseases by studying cells and tissues under a microscope.
PDQ
PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.
PET scan (… skan)
A procedure in which a small amount of radioactive glucose (sugar) is injected into a vein, and a scanner is used to make detailed, computerized pictures of areas inside the body where the glucose is used. Because cancer cells often use more glucose than normal cells, the pictures can be used to find cancer cells in the body. Also called positron emission tomography scan.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
pineal gland (PIH-nee-ul ...)
A tiny organ in the cerebrum that produces melatonin. Also called pineal body and pineal organ.
pineoblastoma (PIH-nee-oh-blas-TOH-muh)
A fast growing type of brain tumor that occurs in or around the pineal gland, a tiny organ near the center of the brain.
primitive neuroectodermal tumor (PRIH-muh-tiv NOOR-oh-EK-toh-DER-mul TOO-mer)
One of a group of cancers that develop from the same type of early cells, and share certain biochemical and genetic features. Some primitive neuroectodermal tumors develop in the brain and central nervous system (CNS-PNET), and others develop in sites outside of the brain such as the limbs, pelvis, and chest wall (peripheral PNET). Also called PNET.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
radioactive (RAY-dee-oh-AK-tiv)
Giving off radiation.
radioisotope (RAY-dee-oh-I-suh-tope)
An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.
recover (ree-KUH-ver)
To become well and healthy again.
recur (ree-KER)
To come back or to return.
recurrent cancer (ree-KER-ent KAN-ser)
Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.
risk factor (... FAK-ter)
Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.
scan (skan)
A picture of structures inside the body. Scans often used in diagnosing, staging, and monitoring disease include liver scans, bone scans, and computed tomography (CT) or computerized axial tomography (CAT) scans and magnetic resonance imaging (MRI) scans. In liver scanning and bone scanning, radioactive substances that are injected into the bloodstream collect in these organs. A scanner that detects the radiation is used to create pictures. In CT scanning, an x-ray machine linked to a computer is used to produce detailed pictures of organs inside the body. MRI scans use a large magnet connected to a computer to create pictures of areas inside the body.
scanner (SKA-ner)
In medicine, an instrument that takes pictures of the inside of the body.
SPECT
A special type of computed tomography (CT) scan in which a small amount of a radioactive drug is injected into a vein and a scanner is used to make detailed images of areas inside the body where the radioactive material is taken up by the cells. SPECT can give information about blood flow to tissues and chemical reactions (metabolism) in the body. Also called single-photon emission computed tomography.
spectroscopy (spek-TROS-koh-pee)
The study of the amount of light that is taken up, given off, or scattered (reflected) by an object. Spectroscopy breaks down light and measures different wavelengths of visible and non-visible light. In medicine, different types of spectroscopy are being used to study tissues and to help make a diagnosis.
spinal column (SPY-nul KAH-lum)
The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spinal column encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spine, and vertebral column.
spinal cord (SPY-nul kord)
A column of nerve tissue that runs from the base of the skull down the back. It is surrounded by three protective membranes, and is enclosed within the vertebrae (back bones). The spinal cord and the brain make up the central nervous system, and spinal cord nerves carry most messages between the brain and the rest of the body.
stage (stayj)
The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom (SIMP-tum)
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
syndrome (SIN-drome)
A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.
tailbone (TAYL-bone)
The small bone at the bottom of the spine. It is made up of 3-5 fused bones. Also called coccyx.
tissue (TIH-shoo)
A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer)
An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
vomit (VAH-mit)
To eject some or all of the contents of the stomach through the mouth.
x-ray (EX-ray)
A type of radiation used in the diagnosis and treatment of cancer and other diseases. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.

Table of Links

1http://www.cancer.gov/cancertopics/pdq/treatment/adultbrain/Patient
2http://www.cancer.gov/cancertopics/pdq/treatment/child-CNS-ATRT/patient
3http://www.cancer.gov/cancertopics/pdq/treatment/childCNSembryonal/patient/#Sec
tion_16
4http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=43707&tt=1&a
mp;format=1&cn=1
5http://www.cancer.gov/clinicaltrials
6http://www.cancer.gov/cancertopics/pdq/treatment/lateeffects/Patient
7http://www.cancer.gov/cancertopics/pdq/treatment/childCNSembryonal/patient/#Sec
tion_238
8http://cancer.gov/clinicaltrials
9http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=42439&tt=1&a
mp;format=1&cn=1
10http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=562072&tt=1&
amp;format=1&cn=1
11http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=586259&tt=1&
amp;format=1&cn=1
12http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=42431&tt=1&a
mp;format=1&cn=1
13http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=586168&tt=1&
amp;format=1&cn=1
14http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=612114&tt=1&
amp;format=1&cn=1
15http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=608167&tt=1&
amp;format=1&cn=1
16http://www.cancer.gov/cancertopics/wyntk/brain
17http://www.pbtc.org/public/gen_info.htm
18http://www.cancer.gov/cancertopics/wyntk/overview
19http://www.cancer.gov/cancertopics/types/childhoodcancers
20http://www.curesearch.org
21http://www.cancer.gov/cancertopics/aya
22http://www.cancer.gov/cancertopics/youngpeople
23http://www.cancer.gov/cancertopics/factsheet/NCI/children-adolescents
24http://www.cancer.gov/cancertopics/understandingcancer/cancer
25http://www.cancer.gov/cancertopics/factsheet/Detection/staging
26http://www.cancer.gov/cancertopics/coping
27http://www.cancer.gov/cancertopics/literature
28http://dccps.nci.nih.gov/ocs/resources.html
29https://cissecure.nci.nih.gov/livehelp/welcome.asp
30http://cancer.gov
31https://cissecure.nci.nih.gov/ncipubs