Extracranial Germ Cell Tumors Treatment

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

A germ cell is a type of cell that forms as a fetus develops. These cells later become sperm in the testicles or eggs in the ovaries.

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 (tailbone).
• Retroperitoneum (area in the back of the abdomen behind the tissue that lines the abdominal wall and covers most of the organs in the abdomen).
• Mediastinum (area between the lungs).
• Head and neck.

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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), retroperitoneum (the back wall of the abdomen), and the head and neck.

Extracranial germ cell tumors are most common in adolescents, with rates in this age group lower for females than males.

For information on intracranial (inside the brain) germ cell tumors, see Childhood Central Nervous System Germ Cell Tumors Treatment.

Childhood extracranial germ cell tumors may be benign or malignant.

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

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

Malignant extracranial germ cell tumors are tumors that form outside the brain. They are gonadal or extragonadal.

There are three types of extracranial germ cell tumors.

Extracranial germ cell tumors are also grouped into teratomas, malignant germ cell tumors, and mixed germ cell tumors:

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

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

A risk factor is anything that increases the chance of getting a disease. Not every child with one or more of these risk factors will develop an extracranial germ cell tumor. And it will develop in some children who don't have a known risk factor.

Possible risk factors for extracranial germ cell tumors include:

• Having certain genetic syndromes:
  • Klinefelter syndrome may increase the risk of germ cell tumors in the mediastinum.
  • Swyer syndrome may increase the risk of gonadoblastoma and seminoma.
  • Turner syndrome may increase the risk of gonadoblastoma and dysgerminoma.
• Having an undescended testicle may increase the risk of testicular cancer.
• Having an ovary or testicle that has not formed normally (gonadal dysgenesis) may increase the risk of gonadoblastoma.

Talk with your child's doctor if you think your child may be at risk.

Signs of childhood extracranial germ cell tumors depend on where the tumor formed in the body.

Different tumors may cause the following signs and symptoms. Other conditions may cause these same signs and symptoms. It's important to check with your child's doctor if your child has:

• A lump in the neck, abdomen, or lower back.
• A painless lump in the testicle.
• Pain in the abdomen or back.
• Respiratory distress, chest pain, or cough.
• Fever.
• Constipation.
• Trouble urinating.
• Early puberty.
• In females, no menstrual periods or unusual vaginal bleeding.

Imaging studies and blood tests are used to diagnose childhood extracranial germ cell tumors.

If your child has symptoms that suggest they have an extracranial germ cell tumor, their doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child's personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with an extracranial germ cell tumor, the results of these tests will help you and your child's doctor plan treatment.

The tests used to diagnose extracranial germ cell tumors may include:

• 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.

  Some malignant germ cell tumors release tumor markers. The following tumor markers may be used to detect extracranial germ cell tumors:

  • Alpha-fetoprotein (AFP).
  • Beta-human chorionic gonadotropin (beta-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, including lactate dehydrogenase. 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, 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.
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  Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
  

• 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).
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  Magnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
  

• 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.
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  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. Sometimes an incisional biopsy or needle biopsy is done before surgery to remove a sample of tissue. Sometimes the tumor is removed during surgery and then a sample of tissue is removed from the tumor.

  The following tests may be done on the sample of tissue that is removed:

  • Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a sample of tissue are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in certain chromosomes may be a sign of cancer. Cytogenetic analysis is used to help diagnose cancer, plan treatment, or find out how well treatment is working.
  • 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.

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

The prognosis and treatment options depend on the following:

• The child's age and general health.
• The stage of the cancer (whether it has spread to nearby areas, lymph nodes, or to other places in the body).
• Where the tumor first began to grow.
• The type of germ cell tumor.
• How well the tumor responds to treatment.
• Whether the child has gonadal dysgenesis.
• Whether the tumor can be completely removed by surgery.
• 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 brain or lymph nodes. 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 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.
• Fertility specialist.

The following types of 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. Learn more at Clinical Trials Information for Patients and Caregivers.

Treatment for childhood extracranial germ cell tumors may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit 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:

• Physical problems, such as infertility, trouble hearing and kidney problems.
• Changes in mood, feelings, thinking, learning, or memory.
• Second cancers (new types of cancer), such as leukemia or malignant melanoma.

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. 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 check-ups. Some tests that were done to diagnose or stage 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).

For childhood extracranial germ cell tumors, follow-up may include regular physical exams, tumor marker tests, and imaging tests such as CT scan, MRI or chest x-ray.

Dysgerminomas

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of newly diagnosed stage I dysgerminomas of the ovary may include the following:

• Surgery (unilateral salpingo-oophorectomy) followed by observation. Chemotherapy may be given if tumor marker levels do not decrease after surgery or the tumor comes back.
• A clinical trial of a new regimen of surgery followed by observation.

Treatment of newly diagnosed stages II–IV dysgerminomas of the ovary may include the following:

• Surgery (unilateral salpingo-oophorectomy) followed by chemotherapy.
• Chemotherapy to shrink the tumor, followed by surgery (unilateral salpingo-oophorectomy).

Nongerminomas

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of newly diagnosed nongerminomas of the ovary, such as yolk sac tumors, mixed germ cell tumors, choriocarcinoma, and embryonal carcinomas, in young girls may include the following:

• Surgery followed by observation for stage I tumors.
• Surgery followed by chemotherapy for stage I–IV tumors.
• A clinical trial of a new regimen of surgery followed by observation for stage I tumors or chemotherapy for stage II–IV.

Treatment of newly diagnosed nongerminomas of the ovary in adolescents and young women may include the following:

• Surgery and chemotherapy for stage I–IV tumors.
• A clinical trial of a new regimen of surgery followed by observation for stage I or chemotherapy for stage II–IV.

Treatment of newly diagnosed nongerminomas of the ovary that cannot be removed by primary surgery without risk to nearby tissue may include the following:

• Biopsy followed by chemotherapy and surgery.

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