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Childhood Extracranial Germ Cell Tumors Treatment (PDQ)

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General Information About Childhood Extracranial Germ Cell Tumors
Stages of Childhood Extracranial Germ Cell Tumors
Recurrent Childhood Extracranial Germ Cell Tumors
Treatment Option Overview
Treatment Options for Childhood Extracranial Germ Cell Tumors
To Learn More About Childhood Cancer
About This PDQ Summary

General Information About Childhood Extracranial Germ Cell Tumors

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:

Extracranial germ cell tumor; drawing shows parts of the body where extracranial germ cell tumors may form, including the mediastinum (the area between the lungs), retroperitoneum (the area behind the abdominal organs), sacrum, coccyx, testicles (in males), and ovaries (in females). Also shown are the heart and peritoneum.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).

Extracranial germ cell tumors are most common in adolescents 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:

Mature Teratomas

Mature teratomas are the most common type of extracranial germ cell tumor. Mature teratomas are benign tumors and not likely to become cancer. They usually occur in the sacrum or coccyx (bottom part of the spine) in newborns or in the ovaries of girls at the start of puberty. The cells of mature teratomas look almost like normal cells under a microscope. Some mature teratomas release enzymes or hormones that cause signs and symptoms of disease.

Immature Teratomas

Immature teratomas also usually occur in the sacrum or coccyx (bottom part of the spine) in newborns or the ovaries of girls at the start of puberty. Immature teratomas have cells that look very different from normal cells under a microscope. Immature teratomas may be cancer. They often have several different types of tissue in them, such as hair, muscle, and bone. Some immature teratomas release enzymes or hormones that cause signs and symptoms of disease.

Malignant Germ Cell Tumors

Malignant germ cell tumors are cancer. There are two main types of malignant germ cell tumors:

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

Malignant extracranial germ cell tumors are gonadal or extragonadal.

Gonadal Germ Cell Tumors

Gonadal germ cell tumors form in the testicles in boys or ovaries in girls.

Testicular Germ Cell Tumors

Testicular germ cell tumors are divided into two main types, seminoma and nonseminoma.

Testicular germ cell tumors usually occur before the age of 4 years or in adolescents and young adults. Testicular germ cell tumors in adolescents and young adults are different from those that form in early childhood.

Boys older than 14 years with testicular germ cell tumors are treated in pediatric cancer centers, but the treatment is much like the treatment used in adults. (See the PDQ summary on Testicular Cancer Treatment for more information.)

Ovarian Germ Cell Tumors

Ovarian germ cell tumors are more common in adolescent girls and young women. Most ovarian germ cell tumors are benign teratomas. Sometimes immature teratomas, dysgerminomas, yolk sac tumors, and mixed germ cell tumors (cancer) occur. (See the PDQ summary on Ovarian Germ Cell Tumors Treatment for more information.)

Extragonadal Extracranial Germ Cell Tumors

Extragonadal extracranial germ cell tumors form in areas other than the brain, testicles, or ovaries.

Most extragonadal extracranial germ cell tumors form along the midline of the body. This includes the following:

In younger children, extragonadal extracranial germ cell tumors usually occur at birth or in early childhood. Most of these tumors are teratomas in the sacrum or coccyx.

In older children, adolescents, and young adults, extragonadal extracranial germ cell tumors are often in the mediastinum.

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:

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:

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:

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

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

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

Stages of Childhood Extracranial Germ Cell Tumors

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:

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:

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.

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.

Childhood nonseminoma testicular germ cell tumors

Childhood ovarian germ cell tumors

There are two types of stages used for childhood ovarian germ cell tumors. The following stages are from the Children's Oncology Group:

The following stages are from the International Federation of Gynecology and Obstetrics (FIGO):

Childhood extragonadal extracranial germ cell tumors

Recurrent Childhood Extracranial Germ Cell Tumors

Recurrent childhood extracranial germ cell tumor is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body.

The number of patients who have tumors that come back is small. Most recurrent germ cell tumors come back within three years of surgery. About half of the teratomas that recur in the sacrum or coccyx are malignant, so follow-up is important.

Treatment Option Overview

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:

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:

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:

Surgery

Surgery to completely remove the tumor is done whenever possible. If the tumor is very large, chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. A goal of surgery is to keep reproductive function. The following types of surgery may be used:

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy 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.

Observation

Observation is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. For childhood extracranial germ cell tumors, this includes physical exams, imaging tests, and tumor marker tests.

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). Combination chemotherapy is treatment using more than one anticancer drug.

The way the chemotherapy is given depends on the type and stage of the cancer being treated.

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.

High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant 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.

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:

The way the radiation therapy is given depends on the type of cancer and whether it has come back. External radiation therapy is being studied for the treatment of childhood extracranial germ cell tumors that have come back.

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.

Treatment Options for Childhood Extracranial Germ Cell Tumors

Mature and Immature Teratomas

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

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

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

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.

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.

Malignant Gonadal Germ Cell Tumors

Malignant Testicular Germ Cell Tumors

Treatment of malignant testicular germ cell tumors may include the following:

For boys younger than 15 years:

For boys 15 years and older:

Malignant testicular germ cell tumors in boys 15 years and older are treated differently than they are in young boys. Surgery may include removal of lymph nodes in the abdomen. (See the PDQ summary on Testicular Cancer Treatment for more information.)

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.

Malignant Ovarian Germ Cell Tumors

Dysgerminomas

Treatment of stage I dysgerminomas in young girls may include the following:

Treatment of stages II–IV dysgerminomas in young girls may include the following:

Nongerminomas

Treatment of stage I nongerminomas in young girls may include the following:

Treatment of stages II–IV nongerminomas in young girls may include the following:

The treatment for adolescents and young adults with ovarian germ cell tumor is much like the treatment for adults. (See the PDQ treatment summary on Ovarian Germ Cell Tumors for more information.)

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.

Malignant Extragonadal Extracranial Germ Cell Tumors

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

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

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.

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:

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

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 Cancer

For more information from the National Cancer Institute about childhood extracranial germ cell tumors, see the following.

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

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of childhood extracranial germ cell tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials are listed in PDQ and can be found online at NCI's website. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

PDQ Pediatric Treatment Editorial Board. PDQ Childhood Extracranial Germ Cell Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/extracranial-germ-cell/patient/germ-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389180]

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Physicians version: CDR0000062854
Date last modified: 2016-08-12

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Dr. G. Quade
This page was last modified on Thursday, 16-Nov-2017 09:55:23 CET
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