Germ cells are special cells in a developing embryo (fetus) that become the testicles in boys and the ovaries in girls. Rarely, during development of the embryo, these cells travel to other areas of the body and become cancerous. Cancer occurs when cells in the body become abnormal and multiply without control or order. The cells then form a mass of tissue, called a tumor. A germ cell tumor can be benign (noncancerous) or malignant (cancerous). Some places that germ cells may travel to include the chest, abdomen, or brain.

This summary covers germ cell tumors that are extracranial (occurring in places other than the brain) and extragonadal (occurring outside of the reproductive organs). Extracranial, extragonadal germ cell tumors that occur in early childhood generally begin in the sacrum and the coccyx, which are the lowest parts of the spinal column. Extracranial, extragonadal germ cell tumors in people in their teen years and young adulthood are often located in the chest.

For information on a germ cell tumor in the brain, read Cancer.Net Guide to Central Nervous System Tumors, Childhood.

For more information on a germ cell tumor in the reproductive organs, read Cancer.Net's Guide to Ovarian Cancer and Guide to Testicular Cancer.

Statistics

Extracranial, extragonadal germ cell tumors are rare. Extracranial germ cell tumors, including those that occur in the reproductive organs, account for about 3% of all cancers in children younger than 15 and about 14% in children age 15 to 19. More than 60% of childhood germ cell tumors are extragonadal and most commonly occur in the sacrum and coccyx, chest, and abdomen.

Cancer statistics should be interpreted with caution. It is not possible to tell a person how long he or she will live with a germ cell tumor. Because survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this tumor.

Source: National Cancer Institute

To learn about the cancer terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: Basic Oncology Terms.

A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

At this time, there are no known risk factors for the development of a germ cell tumor.

A child with an extracranial, extragonadal germ cell tumor may experience the following symptoms. Sometimes, children with this type of tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a germ cell tumor. If you are concerned about a symptom on this list, please talk with your child’s doctor.

• Pain in the affected area
  
  
• Constipation (infrequent or difficult passage of stool)
  
  
• Urinary retention (inability to urinate)
  
  
• Cough and/or difficulty breathing

Doctors use many tests to diagnose a germ cell tumor and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:

• Age and medical condition
• The type of tumor suspected
• Severity of symptoms
• Previous test results

In addition to a physical examination, the following tests may be used to diagnose a germ cell tumor:

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that a germ cell tumor is present, but only a biopsy can make a definite diagnosis and determine if the tumor is benign or cancerous. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). The pathologist will examine the tissue sample for abnormalities in the cells (histologic examination) and for signs of cancer (cytologic examination).

Blood tests/alphafetoprotein (AFP). A germ cell tumor may cause high levels of AFP, which is a protein that increases in the mother’s blood during pregnancy and can be detected by amniocentesis (a test used to monitor the health of a fetus by using a needle inserted into the abdomen and uterus of a pregnant woman to collect amniotic fluid). AFP is also a tumor marker in adults with a germ cell tumor. A tumor marker is a substance found in higher than normal amounts in the blood, urine, or body of people with certain kinds of tumors. The levels of AFP in the blood may be monitored to determine how well a treatment is working.

Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. A tumor produces different echoes of the sound waves than normal tissue. When the sound waves are bounced back to a computer, creating images, the doctor can locate a mass inside the body.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: Newly Diagnosed.

To learn more about what to expect during common diagnostic tests, read Cancer.Net: Tests and Procedures.

Staging is a way of describing a tumor, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of tumors.

According to the National Cancer Institute, the following stages are used for extracranial germ cell tumors:

Stage I: The tumor can be completely surgically removed, and has not spread to other areas of the body.

Stage II: Cancer has spread to surrounding tissue or lymph nodes. Cancer cells may not be able to be completely removed from the surrounding tissue with surgery.

Stage III: Cancer has spread to surrounding tissue, has affected several lymph nodes, is found in fluid in the abdomen, and the entire tumor cannot be removed from the surrounding tissue with surgery.

Stage IV: Cancer has spread to other organs, most commonly the lungs, liver, or brain.

Recurrent: Recurrent cancer is cancer that comes back after treatment. It may recur at the original site of the tumor or in another place.

clinical trials are the standard of care for the treatment of children with cancer. In fact, more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best treatments available) with newer treatments that may be more effective. Cancer in children is rare, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track progress.

To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. Many times, a team of doctors treats a child with cancer. Pediatric cancer centers often have extra support services for children and their families, such as nutritionists, social workers, and counselors. Special activities for kids with cancer may also be available.

The treatment of a germ cell tumor depends on the size and location of the tumor, whether it is cancerous, whether the cancer has spread, and the child’s overall health.

Surgery

The goal of surgery is to remove the tumor, along with some surrounding tissue (called a margin) to make sure that the entire tumor is removed.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

The specific drugs that are commonly used for treating germ cell tumors include cisplatin (Platinol), etoposide (VePesid, Etopophos, Toposar), bleomycin (Blenoxane), and cyclophosphamide (Cytoxan, Neosar).

The medications used to treat cancer are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: During Treatment.

Doctors and scientists are always looking for better ways to treat patients with a germ cell tumor. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating germ cell tumors. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with a germ cell tumor.

To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.

Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.

Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your child’s health-care team. Before treatment begins, talk with your child’s doctor about possible side effects of the specific treatments your child will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.

Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with your doctor about side effects your child experiences during and after treatment. For more information on the most common side effects of cancer and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects, based on ASCO’s curriculum.

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient.

For more information on late effects or long-term side effects, please read the After Treatment section or talk with your child’s doctor.

After treatment for a germ cell tumor ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children treated for cancer, including germ cell tumors, should have life-long, follow-up care.

Generally, children treated for a germ cell tumor are monitored using physical examinations, x-rays, and blood tests for two years after treatment ends to check for possible recurrence. After this time, it is unlikely that the tumor will recur, and the focus of the follow-up changes to potential late side effects of chemotherapy (if given). Because of the effects of the drugs most commonly used to treat these tumors, regular tests of kidney function, lung function, fertility (ability to have children), and blood cell production may be needed.

Based on the type of treatment the child received, the doctor will determine what examinations and tests are needed to check for long-term side effects, such as growth and development abnormalities, organ damage, and the possibility of secondary cancers. Your child’s doctor can recommend the necessary screening tests. Follow-up care should also address the child’s quality of life, including any developmental or emotional concerns. Learn more about Childhood Cancer Survivorship.

The child’s family is encouraged to organize and keep a record of the child’s medical information, so that as the child enters adulthood, he or she has a clear, written history of the diagnosis and details of the treatment given. The doctor’s office can help you compile this, and it should include recommendations from the doctor about the schedule for follow-up care. This information will be valuable to doctors who care for your child during his or her lifetime.

Children who have had a germ cell tumor can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs. 

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: After Treatment.

Research for germ cell tumors is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this current time. Always discuss all diagnostic and treatment options with your child’s doctor.

Combination chemotherapy. Clinical trials are underway to investigate new combinations of chemotherapy to treat extracranial, extragonadal germ cell tumors. For example, one study is evaluating the use of cyclophosphamide with cisplatin, etoposide, and bleomycin in children newly diagnosed with this type of tumor.

Regular communication with your child’s doctor is important in making informed decisions about his or her health care. Consider asking the following questions of your child’s doctor:

• What type of tumor does my child have?
  
  
• Where is it located?
  
  
• Is it cancerous?
  
  
• If so, what stage is the cancer?
  
  
• Can you explain my child’s pathology report to me?
  
  
• What are the treatment options?
  
  
• What treatment do you recommend? Why?
  
  
• What clinical trials are open to my child?
  
  
• What are the possible side effects of this treatment, both in the short term and the long term?
  
  
• How will this treatment affect my child’s daily life? Will my child be able to go to school and perform his or her usual activities?
  
  
• What are the chances that the tumor will recur?
  
  
• What follow-up tests will my child need, and how often will he or she need them?
  
  
• What support services are available to my child? To my family?