General Information About Extragonadal Germ Cell Tumors

Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body.

“Extragonadal” means outside of the gonads (sex organs). When cells that are meant to form sperm in the testicles or eggs in the ovaries travel to other parts of the body, they may grow into extragonadal germ cell tumors. These tumors may begin to grow anywhere in the body but usually begin in organs such as the pineal gland in the brain, in the mediastinum, or in the abdomen.

Extragonadal germ cell tumors can be benign (noncancer) or malignant (cancer). Benign extragonadal germ cell tumors are called benign teratomas. These are more common than malignant extragonadal germ cell tumors and often are very large.

Malignant extragonadal germ cell tumors are divided into two types, nonseminoma and seminoma. Nonseminomas tend to grow and spread more quickly than seminomas. They usually are large and cause symptoms. If untreated, malignant extragonadal germ cell tumors may spread to the lungs, lymph nodes, bones, liver, or other parts of the body.

For information about germ cell tumors in the ovaries and testicles, see the following PDQ summaries:

• Ovarian Germ Cell Tumors Treatment

• Testicular Cancer Treatment

Age and gender can affect the risk of developing extragonadal germ cell tumors.

Anything that increases your chance 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. People who think they may be at risk should discuss this with their doctor. Risk factors for malignant extragonadal germ cell tumors include the following:

• Being male.

• Being age 20 or older.

• Having Klinefelter syndrome.

Possible signs of extragonadal germ cell tumors include chest pain and breathing problems.

Malignant extragonadal germ cell tumors may cause symptoms as they grow into nearby areas. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

• Chest pain.

• Breathing problems.

• Cough.

• Fever.

• Headache.

• Change in bowel habits.

• Feeling very tired.

• Trouble walking.

• Trouble in seeing or moving the eyes.

Imaging and blood tests are used to detect (find) and diagnose extragonadal 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.

• 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.

• Serum tumor marker test: A procedure in which a sample of blood is examined 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. The following three tumor markers are used to detect extragonadal germ cell tumor:
  • Alpha-fetoprotein (AFP).
  • Beta-human chorionic gonadotropin (β-hCG).
  • Lactate dehydrogenase (LDH).
  Blood levels of the tumor markers help determine if the tumor is a seminoma or nonseminoma.

• 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.

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

• Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The type of biopsy used depends on where the extragonadal germ cell tumor is found.
  • Excisional biopsy: The removal of an entire lump of tissue.
  • Incisional biopsy: The removal of part of a lump or sample of tissue.
  • Core biopsy: The removal of tissue using a wide needle.
  • Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.

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

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

• Whether the tumor is nonseminoma or seminoma.

• The size of the tumor and where it is in the body.

• The blood levels of AFP, β-hCG, and LDH.

• Whether the tumor has spread to other parts of the body.

• The way the tumor responds to initial treatment.

• Whether the tumor has just been diagnosed or has recurred (come back).

Stages of Extragonadal Germ Cell Tumors

After an extragonadal germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

The extent or spread of cancer is usually described as stages. For extragonadal germ cell tumors, prognostic groups are used instead of stages. The tumors are grouped according to how well the cancer is expected to respond to treatment. It is important to know the prognostic group in order to plan treatment.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

• Through tissue. Cancer invades the surrounding normal tissue.

• Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.

• Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

The following prognostic groups are used for extragonadal germ cell tumors:

Good prognosis

A nonseminoma extragonadal germ cell tumor is in the good prognosis group if:

• the tumor is in the back of the abdomen; and

• the tumor has not spread to organs other than the lungs; and

• the levels of tumor markers AFP and β-hCG are normal and LDH is slightly above normal.

A seminoma extragonadal germ cell tumor is in the good prognosis group if:

• the tumor has not spread to organs other than the lungs; and

• the level of AFP is normal; β-hCG and LDH may be at any level.

Intermediate prognosis

A nonseminoma extragonadal germ cell tumor is in the intermediate prognosis group if:

• the tumor is in the back of the abdomen; and

• the tumor has not spread to organs other than the lungs; and

• the level of any one of the tumor markers (AFP, β-hCG, or LDH) is more than slightly above normal.

A seminoma extragonadal germ cell tumor is in the intermediate prognosis group if:

• the tumor has spread to organs other than the lungs; and

• the level of AFP is normal; β-hCG and LDH may be at any level.

Poor prognosis

A nonseminoma extragonadal germ cell tumor is in the poor prognosis group if:

• the tumor is in the chest; or

• the tumor has spread to organs other than the lungs; or

• the level of any one of the tumor markers (AFP, β-hCG, or LDH) is high.

Seminoma extragonadal germ cell tumor does not have a poor prognosis group.

Treatment Option Overview

There are different types of treatment for patients with extragonadal germ cell tumors.

Different types of treatments are available for patients with extragonadal 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. 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.

Three types of standard treatment are used:

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. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

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 in the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Surgery

Patients who have benign tumors or tumor remaining after chemotherapy or radiation therapy may need to have surgery.

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 Web site.

High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant is a method 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.

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 clinical trials database.

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. This is sometimes called re-staging.

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 condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

After initial treatment for extragonadal germ cell tumors, blood levels of AFP and other tumor markers continue to be checked to find out how well the treatment is working.

Treatment Options for Extragonadal Germ Cell Tumors

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Benign Teratoma

Treatment of benign teratomas is surgery.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with benign teratoma.

Seminoma

Treatment of seminoma extragonadal germ cell tumors may include the following:

• Radiation therapy for small tumors in one area, followed by watchful waiting if there is tumor remaining after treatment.

• Chemotherapy for larger tumors or tumors that have spread. If a tumor smaller than 3 centimeters remains after chemotherapy, watchful waiting follows. If a larger tumor remains after treatment, surgery or watchful waiting follow.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with extragonadal seminoma.

Nonseminoma

Treatment of nonseminoma extragonadal germ cell tumors may include the following:

• Combination chemotherapy followed by surgery to remove any remaining tumor.

• A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with malignant extragonadal non-seminomatous germ cell tumor.

Recurrent or Refractory Extragonadal Germ Cell Tumors

Treatment of extragonadal germ cell tumors that are recurrent (come back after being treated) or refractory (do not get better during treatment) may include the following:

• Chemotherapy.

• A clinical trial of high-dose chemotherapy with stem cell transplant.

• A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent extragonadal germ cell tumor.

To Learn More About Extragonadal Germ Cell Tumors

For more information from the National Cancer Institute about extragonadal germ cell tumors, see the Extragonadal Germ Cell Tumor Home Page.

For general cancer information and other resources from the National Cancer Institute, see the following:

• What You Need to Know About™ Cancer - An Overview

• Understanding Cancer Series: Cancer

• Staging: Questions and Answers

• Chemotherapy and You: Support for People With Cancer

• Radiation Therapy and You: Support for People With Cancer

• Coping with Cancer

• Support and Resources

• Cancer Library

• Information For Survivors/Caregivers/Advocates

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Changes to This Summary (06/13/2008)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:

• Text describing the way cancer spreads in the body was added to the Stages section.

• Information about patients taking part in clinical trials and about follow-up tests was added to the Treatment Option Overview section.

• Links to ongoing clinical trials listed in NCI’s PDQ Cancer Clinical Trials Registry were added to the Treatment Options section.

• A new section called “To Learn More” was added. It includes links to more information about this type of cancer and about cancer in general.

• The “Get More Information from NCI” section (originally called “To Learn More”) was revised.

2004-11-22 Last Modified: 2008-06-13