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:
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.
Extracranial germ cell tumors may be benign (noncancer) or malignant (cancer).
Malignant extracranial germ cell tumors are tumors that form outside the brain. They are gonadal or extragonadal.
Gonadal germ cell tumors form in the gonads (testicles and ovaries).
Testicular germ cell tumors usually occur before the age of 5 years or in adolescents and young adults. Testicular germ cell tumors in adolescents (11 years and older) and young adults are different from those that form in early childhood.
Extragonadal extracranial germ cell tumors form in areas of the body other than the brain or gonads (testicles and ovaries).
Most extragonadal extracranial germ cell tumors form along the midline of the body. This includes the following:
In children younger than 11 years, extragonadal extracranial germ cell tumors usually occur at birth or in early childhood. Most of these tumors are benign teratomas in the sacrum or coccyx.
In older children, adolescents, and young adults (11 years and older), extragonadal extracranial germ cell tumors are often in the mediastinum.
Extracranial germ cell tumors are also grouped into teratomas, malignant germ cell tumors, and mixed germ cell tumors:
There are two main types of teratomas:
Malignant germ cell tumors are cancer. There are two main types of malignant germ cell tumors:
Mixed germ cell tumors are made up of at least two types of malignant germ cell tumor. They can form in the ovary, testicle, or other areas of the body.
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:
Talk with your child's doctor if you think your child may be at risk.
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:
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:
Some malignant germ cell tumors release tumor markers. The following tumor markers may be used to detect extracranial germ cell tumors:
The following tests may be done on the sample of tissue that is removed:
The prognosis and treatment options depend on the following:
The prognosis for childhood extracranial germ cell tumors, especially ovarian germ cell tumors, is good.
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.
Cancer can spread through tissue, the lymph system, and the blood:
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.
The following stages are from the Children's Oncology Group.
In stage I, the cancer is found in the testicle only. The testicle and spermatic cord are completely removed by surgery and all of the following are true:
In stage II, the testicle and spermatic cord are removed by surgery and one of the following is true:
Cancer has not spread to the lymph nodes.
In stage III, one of the following is true:
In stage IV, the cancer has spread to other parts of the body, such as the liver, lung, bone, and brain.
In males older than 15 years, there are only stage I tumors and metastatic tumors. For more information about staging used for testicular germ cell tumors in adolescents and young adults 11 years and older, see Testicular Cancer Treatment.
Two staging systems are used for ovarian germ cell tumors: Children's Oncology Group and the International Federation of Gynecology and Obstetrics (FIGO).
The following stages are from the Children's Oncology Group.
In stage I, the tumor in the ovary is completely removed by surgery and all of the following are true:
In stage II, the tumor in the ovary is completely removed by surgery and a biopsy is done before surgery and one of the following is true:
Cancer cells are not found in fluid taken from the abdomen. Cancer is not seen in lymph nodes or tissue that lines the abdomen and cancer is not found in tissue samples taken during a biopsy.
In stage III, there is tumor in the ovary and one of the following is true:
In stage III-X, the tumor can be described as stage I or stage II, except:
In stage IV, one of the following is true:
The following stages are from the International Federation of Gynecology and Obstetrics (FIGO).
In stage I, cancer is found in one or both of the ovaries and has not spread. Stage I is divided into stage IA, stage IB, and stage IC.
In stage II, cancer is found in one or both ovaries and has spread into other areas of the pelvis, or primary peritoneal cancer is found. Stage II is divided into stage IIA and stage IIB.
In stage III, cancer is found in one or both ovaries or primary peritoneal cancer is found. Cancer has spread outside the pelvis to other parts of the abdomen and/or to lymph nodes at the back of the abdomen. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC.
Stage IV is divided into stage IVA and IVB.
The following stages are from the Children's Oncology Group.
In stage I, the tumor is completely removed by surgery and all of the following are true:
In stage II, cancer is not completely removed by surgery and one of the following is true:
Cancer cells are not found in fluid taken from the abdomen. There is no sign of cancer in lymph nodes in the abdomen, pelvis, or chest on a CT scan or MRI.
In stage III, one of the following is true:
In stage IV, the cancer has spread to other parts of the body, such as the liver, lung, bone, or brain.
Recurrent childhood extracranial germ cell tumor is cancer that has come back after it has been treated. The cancer may come back in the same place or in other parts of the body.
Most germ cell tumors do not recur (come back) after treatment. If they do recur, they usually come back within 3 years of surgery. About half of the teratomas that recur in the sacrum or coccyx are cancer, so follow-up is important.
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.
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:
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:
After 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 surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. For children with extracranial germ cell tumors, this includes physical exams, imaging tests, and tumor marker tests.
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). Systemic chemotherapy is used to treat extracranial germ cell tumors.
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.
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 or a donor 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.
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. External radiation therapy is being studied for the treatment of childhood extracranial germ cell tumors that have come back.
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:
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.
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.
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.
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.
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed mature teratomas includes the following:
Treatment of newly diagnosed immature teratomas includes the following:
Sometimes a mature or immature teratoma also has malignant cells. A teratoma with malignant cells may need to be treated differently.
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.
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed malignant testicular germ cell tumors may include the following:
For boys younger than 11 years:
For boys 11 years and older:
Malignant testicular germ cell tumors in boys 11 years and older are treated differently than they are in young boys. For more information, see Testicular Cancer Treatment.
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.
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:
Treatment of newly diagnosed stages II–IV dysgerminomas of the ovary may include the following:
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:
Treatment of newly diagnosed nongerminomas of the ovary in adolescents and young women may include the following:
Treatment of newly diagnosed nongerminomas of the ovary that cannot be removed by primary surgery without risk to nearby tissue 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.
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed childhood malignant extragonadal extracranial germ cell tumors in young children may include the following:
In addition to stage of the disease, treatment of malignant extragonadal extracranial germ cell tumors also depends on where the tumor formed in the body:
Treatment of newly diagnosed childhood malignant extragonadal extracranial germ cell tumors in adolescents and young adults 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.
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of recurrent childhood extracranial germ cell tumors 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.
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, visit:
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.
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.
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 ("Updated") 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.
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 can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
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]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.