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Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®)

Patient Version
Last Modified: 05/18/2012

Treatment Options for Childhood Central Nervous System Embryonal Tumors

Newly Diagnosed Childhood Medulloblastoma
Newly Diagnosed Childhood Pineoblastoma and Pineal Parenchymal Tumors
Newly Diagnosed Childhood Central Nervous System Primitive Neuroectodermal Tumors
Newly Diagnosed Childhood Medulloepithelioma and Ependymoblastoma
Recurrent 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 1. 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 2.

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 3 and childhood pineal parenchymal 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 2.

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 5. 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 2.

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 6 and childhood medulloepithelioma 7. 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 2.

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 8. 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 2.



Glossary Terms

3-dimensional conformal radiation therapy (3-dih-MEN-shuh-nul kun-FOR-mul RAY-dee-AY-shun THAYR-uh-pee)
A procedure that uses a computer to create a 3-dimensional picture of the tumor. This allows doctors to give the highest possible dose of radiation to the tumor, while sparing the normal tissue as much as possible. Also called 3-dimensional radiation therapy and 3D-CRT.
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.
central nervous system (SEN-trul NER-vus SIS-tem)
The brain and spinal cord. Also called CNS.
chemotherapy (KEE-moh-THAYR-uh-pee)
Treatment with drugs that kill cancer cells.
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.
CNS
The brain and spinal cord. Also called central nervous system.
combination chemotherapy (KOM-bih-NAY-shun KEE-moh-THAYR-uh-pee)
Treatment using more than one anticancer drug.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
drug (drug)
Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.
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.
high-dose chemotherapy (hy-dose kee-moh-THAYR-uh-pee)
An intensive drug treatment to kill cancer cells, but that also destroys the bone marrow and can cause other severe side effects. High-dose chemotherapy is usually followed by bone marrow or stem cell transplantation to rebuild the bone marrow.
locally recurrent cancer (LOH-kuh-lee ree-KER-ent KAN-ser)
Cancer that has recurred (come back) at or near the same place as the original (primary) tumor, usually after a period of time during which the cancer could not be detected.
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).
parenchyma (puh-REN-kih-muh)
The essential or functional elements of an organ.
pineal organ (PIH-nee-ul OR-gun)
A tiny organ in the cerebrum that produces melatonin. Also called pineal body and pineal gland.
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.
radiation therapy (RAY-dee-AY-shun THAYR-uh-pee)
The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.
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.
schedule (SKEH-jool)
In a clinical setting, the step-by-step plan for how patients are to be treated; for example, the drug or type of radiation therapy that is to be given, the method by which it is to be given, the amount of time between courses, and the total length of treatment.
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.
standard therapy (... THAYR-uh-pee)
Treatment that experts agree is appropriate, accepted, and widely used. Also called best practice, standard medical care, and standard of care.
stem cell rescue (stem sel REH-skyoo)
A method of replacing immature blood-forming cells that were destroyed by treatment with anticancer drugs. The stem cells help the bone marrow recover and make healthy blood cells. Stem cell rescue is usually done using the patient’s own stem cells that were saved before treatment.
stereotactic radiation therapy (STAYR-ee-oh-TAK-tik RAY-dee-AY-shun THAYR-uh-pee)
A type of external radiation therapy that uses special equipment to position the patient and precisely deliver radiation to a tumor. The total dose of radiation is divided into several smaller doses given over several days. Stereotactic radiation therapy is used to treat brain tumors and other brain disorders. It is also being studied in the treatment of other types of cancer, such as lung cancer. Also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.
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.
targeted therapy (TAR-geh-ted THAYR-uh-pee)
A type of treatment that uses drugs or other substances, such as monoclonal antibodies, to identify and attack specific cancer cells. Targeted therapy may have fewer side effects than other types of cancer treatments.
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.

Table of Links

1http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=42439&tt=1&a
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2http://www.cancer.gov/clinicaltrials
3http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=562072&tt=1&
amp;format=1&cn=1
4http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=586259&tt=1&
amp;format=1&cn=1
5http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=42431&tt=1&a
mp;format=1&cn=1
6http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=586168&tt=1&
amp;format=1&cn=1
7http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=612114&tt=1&
amp;format=1&cn=1
8http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=608167&tt=1&
amp;format=1&cn=1