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Childhood Central Nervous System Germ Cell Tumors Treatment (PDQ®)

Last modified: 2013-08-01
Last downloaded: 2013-09-03

General Information About Childhood Central Nervous System Germ Cell Tumors

Childhood central nervous system (CNS) germ cell tumors form from germ cells.

This summary is about germ cell tumors that start in the central nervous system (brain and spinal cord). Germ cell tumors may also form in other parts of the body. See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for information on germ cell tumors that are extracranial (outside the brain).

The most common place for one or more central nervous system (CNS) germ cell tumors to form is near the pineal gland and in an area that includes the pituitary gland and the tissue just above it. Sometimes germ cell tumors may form in other areas of the brain.

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.

CNS germ cell tumors can occur in both children and adults; treatment for children may be different than treatment for adults. See the following PDQ summaries for more information about the treatment of adults:

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

Childhood CNS germ cell tumors are grouped into germinomas, teratomas, and nongerminomas.

CNS germ cell tumors are usually grouped based on what the cells look like under a microscope. This summary is about the treatment of CNS germ cell tumors in the following groups:

Germinomas

Germinomas are the most common type of CNS germ cell tumor and have a good prognosis.

Teratomas

CNS teratomas may contain different kinds of cells that can grow into tissues, such as hair, muscle, and bone, within the tumor. Teratomas are described as mature or immature, based on how normal the cells look under a microscope. Teratomas are sometimes a mix of mature and immature cells.

Nongerminomas

Some nongerminomas make hormones. The following kinds of tumors are nongerminomas:

The cause of most childhood CNS germ cell tumors is not known.

Possible signs of childhood CNS germ cell tumors include unusual thirst, frequent urination, early puberty, or vision changes.

These and other symptoms may be caused by childhood CNS germ cell tumors. The symptoms may be different depending on where the tumor is, the size of the tumor, and whether the tumor makes hormones. Check with your child’s doctor if you see any of the following problems in your child:

  • Being very thirsty.
  • Making large amounts of clear or almost clear urine.
  • Frequent urination.
  • Bed wetting or getting up at night to urinate.
  • Problems moving eyes or loss of vision.
  • Loss of appetite.
  • Weight loss for no known reason.
  • Early or late puberty.
  • Short stature (being shorter than normal).
  • Headaches.
  • Nausea and vomiting.
  • Fatigue.
  • Doing poorly in school.

Other conditions may cause the same symptoms.

Imaging studies and tests are used to detect (find) and diagnose childhood central nervous system germ cell tumors.

The following tests and procedures may be used:

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.

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.

  • 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 person’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.
  • Visual field exam: An exam to check a person’s field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). The eyes are tested one at a time. The eye not being tested is covered.
  • MRI (magnetic resonance imaging) 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).
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the head, 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.
  • Tumor marker tests: A procedure in which a sample of blood or cerebrospinal fluid (CSF) is checked to measure the amounts of certain substances released into the blood and CSF by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The following tumor markers are used to diagnose some CNS germ cell tumors:
    • Alpha-fetoprotein (AFP).
    • Beta-human chorionic gonadotropin (β-hCG).
    If there are no tumor markers in the blood or CSF, a biopsy of the tumor is done.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. In some cases, the tumor is removed during surgery and a biopsy may be done.
  • 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.
  • Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle into the spinal column. A sample of CSF is viewed under a microscope by a pathologist to check for signs of tumor cells. This procedure is also called an LP or spinal tap.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.
  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
  • Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher- or lower-than-normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The blood will be checked for the levels of hormones made by the pituitary gland and other glands.

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

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

  • The type of germ cell tumor.
  • The level of tumor marker.
  • Where the tumor is in the brain or spinal cord.
  • Whether the cancer has spread within the brain and spinal cord or to other parts of the body.
  • Whether the tumor is newly diagnosed or has recurred (come back) after treatment.

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Stages of Childhood Central Nervous System Germ Cell Tumors

Childhood central nervous system germ cell tumors rarely spread outside of the brain and spinal cord.

Staging is the process used to find out how much cancer there is and if cancer has spread. There is no standard staging system for childhood central nervous system (CNS) germ cell tumors. The treatment plan depends on the type of germ cell tumor and if the tumor has spread within the CNS or to other parts of the body. The information from tests and procedures done to detect (find) childhood CNS germ cell tumors is also used to plan treatment. The tumors are grouped as newly diagnosed or recurrent.


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Recurrent Childhood Central Nervous System Germ Cell Tumors

Childhood central nervous system germ cell tumors may recur (come back) after they have been treated. The tumors usually come back in the same place as the first tumor. The tumor also may come back in other places and/or in the meninges (thin layers of tissue that cover and protect the brain and spinal cord).


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Treatment Option Overview

There are different types of treatment for patients with childhood central nervous system germ cell tumors.

Different types of treatment are available for children with childhood central nervous system germ cell 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 with childhood central nervous system germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

Treatment will be overseen by a pediatric oncologist and/or a radiation 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 childhood central nervous system germ cell tumors and who specialize in certain areas of medicine. These may include the following specialists:

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

Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include:

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

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).

Four types of standard treatment are used:

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

Radiation therapy to the brain can affect growth and development in young children. Certain ways of giving radiation therapy can lessen the damage to healthy brain tissue. For children younger than 3 years, chemotherapy may be given instead. This can 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). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Surgery

Whether surgery to remove the tumor can be done depends on where the tumor is in the brain. Surgery to remove the tumor may cause severe, long-term side effects.

Surgery may be done to remove teratomas and may be used for germ cell tumors that come back. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy 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.

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.

New types of treatment are being tested in clinical trials.

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

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. 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 cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Children whose cancer affected their pituitary gland at diagnosis will usually need to have their blood hormone levels checked. If the blood hormone level is low, replacement hormone medicine is given. Children who had a high tumor marker level (alpha-fetoprotein or beta-human chorionic gonadotropin) at diagnosis, usually need to have their blood tumor marker level checked. If the tumor marker level increases after initial treatment, the tumor may have recurred.


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Treatment Options for Childhood Central Nervous System Germ Cell Tumors

Newly Diagnosed Germinomas

Treatment may include the following:

Newly Diagnosed Teratomas

Treatment may include the following:

Newly Diagnosed Nongerminomas

Treatment of choriocarcinoma, embryonal carcinoma, yolk sac tumor, or mixed germ cell tumor may include the following:

Sometimes the tumor marker level decreases after initial treatment but the tumor can still be seen in imaging tests. Surgery may be done to remove as much of the tumor as possible and do a biopsy.

Recurrent Childhood Central Nervous System Germ Cell Tumors

Treatment may include the following:


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Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood central nervous system germ cell 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.


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To Learn More About Childhood Central Nervous System Germ Cell Tumors

For more information about childhood central nervous system germ cell tumors, see the following:

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


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Changes to This Summary (08/01/2013)

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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Back to TopSource: The National Cancer Institute's Physician Data Query (PDQ®) Cancer Information Summaries (http://www.cancer.gov/cancertopics/pdq)