Childhood Central Nervous System Germ Cell Tumors Treatment
General Information About Childhood Central Nervous System (CNS) Germ Cell Tumors
Key Points
Childhood central nervous system (CNS) germ cell tumors form from germ cells.
There are different types of childhood CNS germ cell tumors.
Germinomas
Nongerminomas
Teratomas
Signs and symptoms of childhood CNS germ cell tumors include unusual thirst, frequent urination, or vision changes.
Imaging studies and other tests are used to help diagnose childhood CNS germ cell tumors.
A biopsy may be done to be sure of the diagnosis of a CNS germ cell tumor.
Certain factors affect prognosis (chance of recovery).
Childhood central nervous system (CNS) germ cell tumors form from germ cells.
Germ cells are the reproductive cells in a fetus. These cells later become sperm in the testicles or unfertilized eggs in the ovaries. Sometimes the germ cells travel to or from other parts of the fetus as it develops and later become germ cell tumors. Most germ cell tumors form in the testes or ovaries. Germ cell tumors that form in the brain or spinal cord are called CNS (central nervous system) germ cell tumors.
CNS germ cell tumors occur most often in people aged 10 to 19 years and more often in males than in females. The most common places for one or more CNS germ cell tumors to form is in the brain near the pineal gland and in an area of the brain that includes the pituitary gland and the tissue just above it. Sometimes germ cell tumors form in other areas of the brain.
EnlargeAnatomy 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.
The cause of most childhood CNS germ cell tumors is not known.
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. For information on germ cell tumors that are extracranial (outside the brain), see Childhood Extracranial Germ Cell Tumors Treatment.
Treatment of CNS germ cell tumors may be different for children and adults. See the following PDQ summaries for information about treatment for adults:
There are different types of childhood CNS germ cell tumors.
Different types of CNS germ cell tumors can form from the germ cells that later become sperm or unfertilized eggs. The type of CNS germ cell tumor that is diagnosed depends on what the cells look like under a microscope and results of laboratory tests that check tumor marker levels.
This summary is about the treatment of several types of CNS germ cell tumors.
Germinomas
Germinomas are the most common type of CNS germ cell tumor and have a good prognosis. Tumor marker levels are not used to diagnose germinomas.
Mixed germ cell tumors are made of more than one kind of germ cell. They may make AFP and beta-hCG.
Teratomas
CNS teratomas are described as mature or immature, based on how normal the cells look under a microscope. Mature teratomas look almost like normal cells under a microscope and are made of different kinds of tissue, such as hair, muscle, and bone. Immature teratomas look very different from normal cells under a microscope and are made of cells that look like fetal cells. Some immature teratomas are a mix of mature and immature cells. Tumor marker levels are not used to diagnose teratomas.
Signs and symptoms of childhood CNS germ cell tumors include unusual thirst, frequent urination, or vision changes.
Whether the tumor or the body make too much of certain hormones.
Signs and symptoms may be caused by childhood CNS germ cell tumors or by other conditions. Check with your child's doctor if your child has any of the following:
Being very thirsty.
Making large amounts of urine that is clear or almost clear.
Frequent urination.
Bed wetting or getting up at night to urinate.
Trouble moving the eyes, trouble seeing clearly, or seeing double.
Imaging studies and other tests are used to help diagnose childhood CNS germ cell tumors.
In addition to asking about your child's personal and family health history and doing a physical exam, your child's doctor may perform the following tests and procedures:
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, reflexes, and senses 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 and without 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 may collect around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the lining around the spinal cord to remove a sample of the CSF. The sample of CSF is checked under a microscope for signs of tumor cells and tested for tumor markers. The amount of protein and glucose in the sample may also be tested. This procedure is also called an LP or spinal tap.EnlargeLumbar 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.
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 or CSF normally by organs and tissues, or at abnormally high levels by tumor cells in the body. Certain substances are linked to specific types of cancer when found at increased levels in the blood or CSF. These are called tumor markers.
The following tumor markers are used to diagnose some CNS germ cell tumors:
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.
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.
A biopsy may be done to be sure of the diagnosis of a CNS germ cell tumor.
If doctors think your child may have a CNS germ cell tumor, a biopsy may be done. For brain tumors, the biopsy can be done by removing part of the skull or making a small hole in the skull and using a needle or surgical device to remove a sample of tissue. Sometimes, when a needle is used, it is guided by a computer to remove the tissue sample. 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 piece of skull is usually put back in place after the procedure.
EnlargeCraniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.
The following test may be done on the sample of tissue that is removed:
Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient's tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
Sometimes the diagnosis can be made based on the results of imaging and tumor marker tests and a biopsy is not needed.
Certain factors affect prognosis (chance of recovery).
The prognosis depends on the following:
The type of germ cell tumor.
The type and level of any tumor markers.
Where the tumor is in the brain or in the 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.
Stages of Childhood CNS Germ Cell Tumors
Key Points
Childhood central nervous system (CNS) germ cell tumors rarely spread outside of the brain and spinal cord.
Sometimes childhood central nervous system germ cell tumors come back after treatment.
Childhood central nervous system (CNS) germ cell tumors rarely spread outside of the brain and spinal cord.
Whether the tumor is newly diagnosed or has recurred (come back) after treatment.
Sometimes childhood central nervous system germ cell tumors come back after treatment.
The tumors usually recur (come back) where they first formed. The tumors may also come back in other places and/or in the meninges (thin layers of tissue that cover and protect the brain and spinal cord).
Treatment Option Overview
Key Points
There are different types of treatment for children with central nervous system (CNS) germ cell tumors.
Children with CNS germ cell tumors should have their treatment planned
by a team of healthcare providers who are experts in treating
cancer in children.
Four types of treatment are used:
Radiation therapy
Chemotherapy
Surgery
High-dose chemotherapy with stem cell rescue
New types of treatment are being tested in clinical
trials.
Treatment for childhood CNS germ cell tumors may cause side effects.
Patients may want to think about taking part in a
clinical trial.
Patients can enter clinical trials before, during, or
after starting their cancer treatment.
Follow-up tests may be needed.
There are different types of treatment for children with central nervous system (CNS) germ cell tumors.
Different types of
treatment are available for children with central nervous system (CNS) 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 people
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 CNS germ cell tumors should have their treatment planned
by a team of healthcare providers who are experts in treating
cancer in children.
Treatment will be overseen by a pediatric oncologist and/or a radiation oncologist. A pediatric oncologist is a doctor who specializes in treating children with cancer. A radiation oncologist specializes in treating cancer with radiation therapy. These doctors work with other pediatrichealthcare providers who
are experts in treating children with CNS germ cell tumors and who specialize
in certain areas of medicine. These may include the following
specialists:
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.
External radiation therapy uses a
machine outside the body to send radiation toward the area of the body with cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. This type of radiation therapy may include the following:
Stereotactic radiosurgery: Stereotactic radiosurgery is a type of external radiation therapy. A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims a single large dose of radiation directly at the tumor. This procedure does not involve surgery. It is also called stereotaxic radiosurgery, radiosurgery, and radiation surgery.
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).
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. After 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 doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, 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 website.
Treatment for childhood CNS germ cell tumors may cause side effects.
Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer
treatment may include the following:
Some late effects may be treated or controlled. Talk with your child's doctors about the possible late
effects caused by some treatments. For more information, see Late Effects of Treatment
for Childhood Cancer.
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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
As your child goes through treatment, they will have follow-up tests or checkups. Some of the tests that were done to diagnose the cancer may be repeated 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.
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).
Children whose cancer affected their pituitary gland when the cancer was diagnosed 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) when the cancer was diagnosed usually need to have their blood tumor marker level checked. If the tumor marker level increases after initial treatment, the tumor may have recurred.
Surgery. If a mass remains after chemotherapy that continues to grow and tumor marker levels are normal (called growing teratoma syndrome), surgery may be needed to check if the mass is part teratoma, fibrosis, or a growing tumor.
If the mass is a mature teratoma or fibrosis, radiation therapy is given.
If the mass is a growing tumor, other treatments may be given.
A clinical trial of chemotherapy with radiation therapy to treat patients with CNS nongerminomas that have not spread.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
To Learn More About Childhood CNS Germ Cell Tumors
For more information about childhood central nervous system germ cell tumors, see the following:
