Childhood extracranial germ cell tumors form from germ cells in parts of the body other than the brain.

A germ cell is a type of cell that forms as a fetus (unborn baby) develops. These cells later become sperm in the testicles or eggs in the ovaries. Sometimes while the fetus is forming, germ cells travel to parts of the body where they should not be and grow into a germ cell tumor. The tumor may form before or after birth.

This summary is about germ cell tumors that form in parts of the body that are extracranial (outside the brain). Extracranial germ cell tumors usually form in the following areas of the body:

• Testicles.
• Ovaries.
• Sacrum or coccyx (bottom part of the spine).
• Retroperitoneum (the back wall of the abdomen).
• Mediastinum (area between the lungs).

Figure

Extracranial germ cell tumors form in parts of the body other than the brain. This includes the testicles, ovaries, sacrum (lower part of the spine), coccyx (tailbone), mediastinum (area between the lungs), and retroperitoneum (the back wall of the abdomen). (more...)

Extracranial germ cell tumors are most common in teenagers 15 to 19 years of age.

See the PDQ summary on Childhood Central Nervous System Germ Cell Tumors Treatment for information on intracranial (inside the brain) germ cell tumors.

Childhood extracranial germ cell tumors may be benign or malignant.

Extracranial germ cell tumors may be benign (noncancer) or malignant (cancer).

There are three types of extracranial germ cell tumors.

Extracranial germ cell tumors are grouped into mature teratomas, immature teratomas, and malignant germ cell tumors:

Childhood extracranial germ cell tumors are grouped as gonadal or extragonadal.

Malignant extracranial germ cell tumors are gonadal or extragonadal.

The cause of most childhood extracranial germ cell tumors is unknown.

Having certain inherited disorders can increase the risk of an extracranial germ cell tumor.

Anything that increases your 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.

Possible risk factors for extracranial germ cell tumors include the following:

• Having certain genetic syndromes:

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  Klinefelter syndrome may increase the risk of germ cell tumors in the mediastinum.

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  Swyer syndrome may increase the risk of germ cell tumors in the testicles or ovaries.

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  Turner syndrome may increase the risk of germ cell tumors in the ovaries.

• Having an undescended testicle may increase the risk of developing a testicular germ cell tumor.

Signs of childhood extracranial germ cell tumors depend on the type of tumor and where it is in the body.

Different tumors may cause the following signs and symptoms. Other conditions may cause these same signs and symptoms. Check with a doctor if your child has any of the following:

• A lump in the abdomen or lower back.
• A painless lump in the testicle.
• Pain in the abdomen.
• Fever.
• Constipation.
• In females, no menstrual periods.
• In females, unusual vaginal bleeding.

Imaging studies and blood tests are used to detect (find) and diagnose childhood extracranial germ cell 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. The testicles may be checked for lumps, swelling, or pain. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Serum tumor marker test: A procedure in which a sample of blood is checked to measure the amounts of certain substances released into the blood 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.
  Most malignant germ cell tumors release tumor markers. The following tumor markers are used to detect extracranial germ cell tumors:

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  Alpha-fetoprotein (AFP).

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  Beta-human chorionic gonadotropin (β-hCG).

  For testicular germ cell tumors, blood levels of the tumor markers help show if the tumor is a seminoma or nonseminoma.
• 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.
• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, 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.

  

  Figure

  Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.

• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

  

  Figure

  Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.

• Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.

  

  Figure

  Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

• 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 then a biopsy is done.
  The following tests may be done on the sample of tissue that is removed:

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  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.

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  Immunohistochemistry: A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.

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.
• Where the tumor first began to grow.
• The stage of the cancer (whether it has spread to nearby areas or to other places in the body).
• How well the tumor responds to treatment.
• Whether the tumor can be completely removed by surgery.
• The patient's age and general health.
• Whether the cancer has just been diagnosed or has recurred (come back).

The prognosis for childhood extracranial germ cell tumors, especially ovarian germ cell tumors, is good.

After a childhood extracranial germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread from where the tumor started to nearby areas or to other parts of the body.

The process used to find out if cancer has spread from where the tumor started to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. In some cases, staging may follow surgery to remove the tumor.

The following procedures may be used:

• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the lymph nodes. This procedure is also called nuclear magnetic resonance imaging.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or lymph nodes, 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.
• 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 with cancer and is detected by a scanner.
• Thoracentesis: The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.
• Paracentesis: The removal of fluid from the space between the lining of the abdomen and the organs in the abdomen, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.

The results from tests and procedures used to detect and diagnose childhood extracranial germ cell tumors may also be used in staging.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if an extracranial germ cell tumor spreads to the liver, the cancer cells in the liver are actually cancerous germ cells. The disease is metastatic extracranial germ cell tumor, not liver cancer.

Stages are used to describe the different types of extracranial germ cell tumors.

There are different types of treatment for children with extracranial germ cell tumors.

Different types of treatments are available for children with extracranial 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 extracranial 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, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with extracranial germ cell tumors and who specialize in certain areas of medicine. These may include the following specialists:

• Pediatrician.
• Pediatric surgeon.
• Pediatric hematologist.
• Radiation oncologist.
• Endocrinologist.
• Pediatric nurse specialist.
• Rehabilitation specialist.
• Child life professional.
• Psychologist.
• Social worker.
• Geneticist.

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).

For example, late effects of surgery to remove tumors in the sacrum or coccyx include constipation, loss of bowel and bladder control, and scars.

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:

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 website.

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.

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.

For childhood extracranial germ cell tumors, alpha-fetoprotein (AFP) tests and beta-human chorionic gonadotropin (β-hCG) tests are done to see if treatment is working. Continued high levels of AFP or β-hCG may mean the cancer is still growing. For at least 3 years after surgery, follow-up will include regular physical exams, imaging tests, and tumor marker tests.

Mature and Immature Teratomas

Treatment of mature teratomas that are not in the sacrum or coccyx (bottom part of the spine) includes the following:

• Surgery to remove the tumor followed by observation.

Treatment of immature teratomas that are not in the sacrum or coccyx includes the following:

• Surgery to remove the tumor followed by observation for stage I tumors.
• Surgery to remove the tumor and combination chemotherapy for stage II–IV tumors. It is not known if chemotherapy will help the patient live longer.

Treatment of immature teratomas that are in the sacrum or coccyx includes the following:

• Surgery (removal of the sacrum and coccyx) followed by observation.

Sometimes a mature or immature teratoma also has malignant cells. The teratoma and malignant cells may need to be treated differently.

Regular follow-up exams with imaging tests and the alpha-fetoprotein (AFP) tumor marker test will be done for at least 3 years.

Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood teratoma. 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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

Malignant Gonadal Germ Cell Tumors

Malignant Extragonadal Extracranial Germ Cell Tumors

Treatment of childhood malignant extragonadal extracranial germ cell tumors may include the following:

• Combination chemotherapy to shrink the tumor followed by surgery to remove the sacrum and coccyx (bottom part of the spine) for tumors that are in the sacrum or coccyx.
• Combination chemotherapy to shrink the tumor followed by surgery to remove tumors that are in the mediastinum.
• Biopsy followed by combination chemotherapy to shrink the tumor and surgery to remove tumors that are in the abdomen.
• Surgery to remove the tumor followed by combination chemotherapy for tumors of the head and neck.

Treatment of malignant extragonadal extracranial germ cell tumors in places not already described includes the following:

• Surgery followed by combination chemotherapy.

Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood extragonadal 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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

Recurrent Childhood Malignant Extracranial Germ Cell Tumors

There is no standard treatment for recurrent childhood malignant extracranial germ cell tumors. Treatment depends on the following:

• The type of treatment given when the cancer was diagnosed.
• How the tumor responded to the initial treatment.

Treatment is usually within a clinical trial and may include the following:

• Surgery.
• Surgery followed by combination chemotherapy, for most malignant extracranial germ cell tumors including immature teratomas, malignant testicular germ cell tumors, and malignant ovarian germ cell tumors.
• Surgery for tumors that come back in the sacrum or coccyx (bottom part of the spine), if surgery to remove the sacrum and coccyx was not done when the cancer was diagnosed. Chemotherapy may be given before surgery, to shrink the tumor. If any tumor remains after surgery, radiation therapy may also be given.
• Combination chemotherapy for stage I malignant testicular germ cell tumors and stage I ovarian dysgerminomas.
• High-dose chemotherapy and stem cell transplant.
• Radiation therapy followed by surgery to remove the tumor in the brain for cancer that has spread to the brain.

Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood malignant 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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

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