Key Points for This Section

Childhood extracranial germ cell tumors form from developing sperm or egg cells that travel to parts of the body other than the brain. Childhood extracranial germ cell tumors may be benign or malignant. There are three types of extracranial germ cell tumors. Mature Teratomas Immature Teratomas Malignant Germ Cell Tumors Childhood extracranial germ cell tumors are grouped as gonadal or extragonadal. Gonadal Germ Cell Tumors Extragonadal Extracranial Germ Cell Tumors The cause of most childhood extracranial germ cell tumors is unknown. Having certain inherited disorders can increase the risk of developing an extracranial germ cell tumor. Signs of childhood extracranial germ cell tumors depend on the type of tumor and where it is in the body. Imaging studies and blood tests are used to detect (find) and diagnose childhood extracranial germ cell tumors. Certain factors affect prognosis (chance of recovery) and treatment options.

Childhood extracranial germ cell tumors form from developing sperm or egg cells that travel to parts of the body other than the brain.

As a fetus develops, certain cells form sperm in the testicles or eggs in the ovaries. Sometimes these cells travel to other parts of the body and grow into germ cell tumors. This summary is about germ cell tumors that form in parts of the body that are extracranial (outside the brain). Extracranial germ cell tumors are most common in teenagers 15 to 19 years old.

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, or malignant germ cell tumors:

Mature Teratomas

Mature teratomas are the most common type of extracranial germ cell tumor. The cells of mature teratomas look very much like normal cells. Mature teratomas are benign and not likely to become cancer.

Immature Teratomas

Immature teratomas have cells that look very different from normal cells. They are more likely to become cancer.

Malignant Germ Cell Tumors

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

• Yolk sac tumors: Tumors that make a hormone called alpha-fetoprotein (AFP).
• Germinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (β-hCG).
• Choriocarcinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (β-hCG).

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

Malignant extracranial germ cell tumors are grouped into gonadal and extragonadal.

Gonadal Germ Cell Tumors

Gonadal germ cell tumors form in the testicles or ovaries.

Testicular Germ Cell Tumors

Testicular germ cell tumors usually occur before the age of 4 years or in teenagers and young adults.

Testicular germ cell tumors in teenagers and young adults are different from those that form in early childhood. They are more like testicular cancer in adults. Testicular germ cell tumors are divided into two main types, nonseminoma and seminoma. (See the PDQ summary on Testicular Cancer Treatment ¹ for more information.)

• Nonseminoma: These tumors are usually large and cause symptoms. They tend to grow and spread more quickly than seminomas.
• Seminoma: These tumors make a hormone called beta-human chorionic gonadotropin (β-hCG). They are more sensitive to radiation therapy than nonseminomas.

Boys older than 14 years with testicular germ cell tumors are treated in pediatric cancer centers, but the treatment is similar to that used in adults. (See the PDQ summary on Testicular Cancer Treatment ¹ for more information.)

Ovarian Germ Cell Tumors

Ovarian germ cell tumors form in egg-making cells in an ovary. These tumors are more common in teenage girls and young women. Most ovarian germ cell tumors are benign teratomas. (See the PDQ summary on Ovarian Germ Cell Tumors Treatment ² for more information.)

Extragonadal Extracranial Germ Cell Tumors

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

Most germ cell tumors that are not in the testicles, ovaries, or brain, form along the midline of the body. This includes the following:

• Sacrum (the large, triangle-shaped bone in the lower spine that forms part of the pelvis).
• Coccyx (the small bone at the bottom of the spine, also called the tailbone).
• Mediastinum (the area between the lungs).
• Back of the abdomen.
• Neck.

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, teenagers, 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 developing 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. People who think they may be at risk should discuss this with their doctor. Possible risk factors for extracranial germ cell tumors include the following:

• Having certain genetic syndromes may increase the risk of developing childhood germ cell tumors:
  • Klinefelter syndrome may increase the risk of developing germ cell tumors in the mediastinum.
  • Swyer syndrome may increase the risk of developing germ cell tumors in the testes or ovaries.
• Having an undescended testicle may increase the risk of developing a testicular germ cell tumor.

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 symptoms. A doctor should be consulted if any of these problems occur.

• Most tumors of the sacrum and coccyx can be seen as a lump.
• A testicular tumor may cause a painless lump in the testicles.
• An ovarian germ cell tumor may cause:
  • Pain or a lump in the abdomen.
  • Fever.
  • Constipation.
  • No menstruation.
  • Unusual vaginal bleeding.

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:

• 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.
• 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.

  Most malignant germ cell tumors release tumor markers. The following tumor markers are used to detect extracranial germ cell tumors:

  • Alpha-fetoprotein (AFP).
  • Beta-human chorionic gonadotropin (β-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. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
• Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
• Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
• 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, 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 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. In some cases, the tumor is removed during surgery and then a biopsy is done.

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

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

• The type of germ cell tumor.
• Where the tumor first began to grow.
• The stage of the cancer (whether it has spread to nearby areas or to other places in the body).
• Whether the tumor can be completely removed by surgery.
• The patient's age and general health.
• 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.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

A protein normally produced by a fetus. AFP levels are usually undetectable in the blood of healthy adult men or women (who are not pregnant). An elevated level of AFP suggests the presence of either a primary liver cancer or germ cell tumor. Also called AFP.

A protein made by plasma cells (a type of white blood cell) in response to an antigen (a substance that causes the body to make a specific immune response). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.

Any substance that causes the body to make a specific immune response.

Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.

A hormone normally found in the blood and urine during pregnancy. It may also be produced by some tumor cells. An increased level of beta-human chorionic gonadotropin may be a sign of cancer of the testis, uterus, ovary, liver, stomach, pancreas, or lung. Beta-human chorionic gonadotropin may also be produced in response to certain conditions that are not cancer. Beta-human chorionic gonadotropin is being studied in the treatment of Kaposi sarcoma. Also called ß-hCG.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.

A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.

A malignant, fast-growing tumor that develops from trophoblastic cells (cells that help an embryo attach to the uterus and help form the placenta). Almost all choriocarcinomas form in the uterus after fertilization of an egg by a sperm, but a small number form in a testis or an ovary. Choriocarcinomas spread through the blood to other organs, especially the lungs. They are a type of gestational trophoblastic disease. Also called chorioblastoma, chorioepithelioma, and chorionic carcinoma.

Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.

The small bone at the bottom of the spine. It is made up of 3-5 fused bones. Also called tailbone.

A condition in which stool becomes hard, dry, and difficult to pass, and bowel movements don’t happen very often. Other symptoms may include painful bowel movements, and feeling bloated, uncomfortable, and sluggish.

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.

The study of chromosomes and chromosomal abnormalities.

The process of identifying a disease, such as cancer, from its signs and symptoms.

Outside of the cranium (bones that surround the brain).

A rare cancer that forms in germ cells in the testicle or ovary, or in germ cells that have traveled to areas of the body other than the brain (such as the chest, abdomen, or tailbone). Germ cells are reproductive cells that develop into sperm in males and eggs in females.

The developing offspring from 7 to 8 weeks after conception until birth.

An increase in body temperature above normal (98.6 degrees F), usually caused by disease.

Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.

A type of tumor that begins in the cells that give rise to sperm or eggs. Germ cell tumors can occur almost anywhere in the body and can be either benign or malignant.

The part of the reproductive system that produces and releases eggs (ovary) or sperm (testicle/testis).

One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

A genetic disorder in males caused by having one or more extra X chromosomes. Males with this disorder may have larger than normal breasts, a lack of facial and body hair, a rounded body type, and small testicles. They may learn to speak much later than other children and may have difficulty learning to read and write. Klinefelter syndrome increases the risk of developing extragonadal germ cell tumors and breast cancer.

A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.

The area between the lungs. The organs in this area include the heart and its large blood vessels, the trachea, the esophagus, the thymus, and lymph nodes but not the lungs.

Periodic discharge of blood and tissue from the uterus. From puberty until menopause, menstruation occurs about every 28 days when a woman is not pregnant.

An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.

A group of testicular cancers that begin in the germ cells (cells that give rise to sperm). Nonseminomas are identified by the type of cell in which they begin and include embryonal carcinoma, teratoma, choriocarcinoma, and yolk sac carcinoma.

A part of the body that performs a specific function. For example, the heart is an organ.

An abnormal mass of tissue that forms in germ (egg) cells in the ovary (female reproductive gland in which the eggs are formed). These tumors usually occur in teenage girls or young women, usually affect just one ovary, and can be benign (not cancer) or malignant (cancer). The most common ovarian germ cell tumor is called dysgerminoma.

One of a pair of female reproductive glands in which the ova, or eggs, are formed. The ovaries are located in the pelvis, one on each side of the uterus.

A doctor who identifies diseases by studying cells and tissues under a microscope.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

Having to do with children.

The lower part of the abdomen, located between the hip bones.

An exam of the body to check for general signs of disease.

The likely outcome or course of a disease; the chance of recovery or recurrence.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.

An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.

To come back or to return.

Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.

The large, triangle-shaped bone in the lower spine that forms part of the pelvis. It is made of 5 fused bones of the spine.

A type of cancer of the testicles. Seminomas may spread to the lung, bone, liver, or brain.

A blood test that measures the amount of substances called tumor markers (or biomarkers). Tumor markers are released into the blood by tumor cells or by other cells in response to tumor cells. A high level of a tumor marker may be a sign of cancer.

A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called ultrasonogram.

The male reproductive cell, formed in the testicle. A sperm unites with an egg to form an embryo.

The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spine encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spinal column, and vertebral column.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.

The small bone at the bottom of the spine. It is made up of 3-5 fused bones. Also called coccyx.

A type of germ cell tumor that may contain several different types of tissue, such as hair, muscle, and bone. Teratomas occur most often in the ovaries in women, the testicles in men, and the tailbone in children. Not all teratomas are malignant.

One of two egg-shaped glands inside the scrotum that produce sperm and male hormones. Also called testis.

Cancer that forms in tissues of the testis (one of two egg-shaped glands inside the scrotum that make sperm and male hormones). Testicular cancer usually occurs in young or middle-aged men. Two main types of testicular cancer are seminomas (cancers that grow slowly and are sensitive to radiation therapy) and nonseminomas (different cell types that grow more quickly than seminomas).

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.

A substance that may be found in tumor tissue or released from a tumor into the blood or other body fluids. A high level of a tumor marker may mean that a certain type of cancer is in the body. Examples of tumor markers include CA 125 (in ovarian cancer), CA 15-3 (in breast cancer), CEA (in ovarian, lung, breast, pancreas, and gastrointestinal tract cancers), and PSA (in prostate cancer).

A procedure in which high-energy sound waves are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.

A condition in which one or both testicles fail to move from the abdomen, where they develop before birth, into the scrotum. Undescended testicles may increase the risk for development of testicular cancer. Also called cryptorchidism.

Having to do with the vagina (the birth canal).

A blood vessel that carries blood to the heart from tissues and organs in the body.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.