Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment - Patient Information [NCI]

Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment - Patient Information [NCI]

This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.

Childhood Central Nervous System Embryonal Tumors Treatment

General Information About Childhood Central Nervous System Embryonal Tumors

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. For more information see the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview about the different types of childhood brain and spinal cord tumors.

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 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.

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 (CNS-PNETs) are fast-growing tumors that usually 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. CNS-PNETs may also form in the brain stem or spinal cord.

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 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. Talk with your child's doctor if you think your child may be at risk. Risk factors for CNS embryonal tumors include having the following inherited diseases:

  • Li-Fraumeni syndrome.
  • Nevoid basal cell carcinoma (Gorlin) syndrome.
  • Turcot syndrome.

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. Check with your child's doctor if you see any of the following problems in your child:

  • 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.

    Lumbar puncture; drawing shows a patient lying in a curled position on a table and a spinal needle (a long, thin needle) being inserted into the lower back. Inset shows a close-up of the spinal needle inserted into the cerebrospinal fluid (CSF) in the lower part of the spinal column.

    Lumbar puncture. A patient lies in a curled position on a table. After a small area on the lower back is numbed, a spinal needle (a long, thin needle) is inserted into the lower part of the spinal column to remove cerebrospinal fluid (CSF, shown in blue). The fluid may be sent to a laboratory for testing.
  • SPECTscan (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.

Drawing of a craniotomy showing a section of the scalp that has been pulled back to remove a piece of the skull; the dura covering the brain has been opened to expose the brain. The layer of muscle under the scalp is also shown.

Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.

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

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 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:

  • 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 aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.

    Bone marrow aspiration and biopsy; drawing shows a patient lying face down on a table and a Jamshidi needle (a long, hollow needle) being inserted into the hip bone. Inset shows the Jamshidi needle being inserted through the skin into the bone marrow of the hip bone.

    Bone marrow aspiration and biopsy. After a small area of skin is numbed, a Jamshidi needle (a long, hollow needle) is inserted into the patient's hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.
  • Bone scan: A procedure to check 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 bloodstream. The radioactive material collects in the bones and is detected by a scanner.

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. 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.

Treatment Option Overview

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:

  • Neurosurgeon.
  • Neurologist.
  • Neuropathologist.
  • Neuroradiologist.
  • Rehabilitation specialist.
  • Radiation oncologist.
  • Psychologist.

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 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 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 or intraventricular 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.

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.

Targeted therapy

Targeted therapy uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Signal transduction inhibitor therapy is a type of targeted therapy being studied in the treatment of recurrent childhood medulloblastoma. Signal transduction inhibitors block signals that are passed from one molecule to another inside a cell. Blocking these signals may kill cancer 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 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 child's doctor for clinical trials that are not listed here but may be right for your child.

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. 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.

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 and childhood pineal parenchymal tumor. 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.

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. 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.

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 and childhood medulloepithelioma. 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.

Recurrent Childhood Central Nervous System Embryonal Tumors

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

  • For patients who previously received radiation therapy and chemotherapy, treatment may be chemotherapy and/or stereotactic radiation therapy.
  • For infants and young children who previously received chemotherapy only and have a local recurrence, treatment may be chemotherapy with radiation therapy to the tumor and the area close to it. Surgery to remove the tumor may also be done.
  • For patients who previously received radiation therapy, high-dose chemotherapy and stem cell rescue may be used. It is not known whether this treatment improves survival.
  • For recurrent medulloblastoma, a clinical trial of targeted therapy.

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. 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.

To Learn More About Childhood Central Nervous System Embryonal Tumors

For more information about childhood central nervous system embryonal tumor, see the following:

  • What You Need to Know About™ Brain Tumors
  • Pediatric Brain Tumor Consortium (PBTC)
  • Understanding Cancer Series: Targeted Therapies (Advances in Targeted Therapies)
  • Targeted Cancer Therapies

For more childhood cancer information and other general cancer resources, see the following:

  • What You Need to Know About™ Cancer
  • Childhood Cancers
  • CureSearch for Children's Cancer
  • Late Effects of Treatment for Childhood Cancer
  • Adolescents and Young Adults with Cancer
  • Young People with Cancer: A Handbook for Parents
  • Care for Children and Adolescents with Cancer
  • Understanding Cancer Series: Cancer
  • Cancer Staging
  • Coping with Cancer: Supportive and Palliative Care
  • Questions to Ask Your Doctor About Cancer
  • Cancer Library
  • Information for Survivors/Caregivers/Advocates

Changes to This Summary (02 / 01 / 2013)

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.

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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.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).

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. 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.

Images in the PDQ summaries are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in the PDQ summaries, along with many other cancer-related images, are available in Visuals Online, a collection of over 2,000 scientific images.

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. 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 or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

Last Revised: 2013-02-01


If you want to know more about cancer and how it is treated, or if you wish to know about clinical trials for your type of cancer, you can call the NCI's Cancer Information Service at 1-800-422-6237, toll free. A trained information specialist can talk with you and answer your questions.


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