Key Points for This Section

Extragonadal germ cell tumors form from developing sperm or egg cells that travel from the gonads to other parts of the body. Age and gender can affect the risk of developing extragonadal germ cell tumors. Possible signs of extragonadal germ cell tumors include chest pain and breathing problems. Imaging and blood tests are used to detect (find) and diagnose extragonadal germ cell tumors. Certain factors affect prognosis (chance of recovery) and treatment options.

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 ¹
• Testicular Cancer Treatment ²

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).

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 alpha-fetoprotein.

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.

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

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.

The long, tube-shaped organ in the abdomen that completes the process of digestion. The bowel has two parts, the small bowel and the large bowel. Also called intestine.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is 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.

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 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.

The removal of a tissue sample with a wide needle for examination under a microscope.

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

A surgical procedure in which an entire lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope.

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

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

The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.

Liquid.

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).

A surgical procedure in which a portion of a lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope.

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.

One of a group of enzymes found in the blood and other body tissues and involved in energy production in cells. An increased amount of lactate dehydrogenase in the blood may be a sign of tissue damage and some types of cancer or other diseases. Also called lactic acid dehydrogenase and LDH.

One of a group of enzymes found in the blood and other body tissues, and involved in energy production in cells. An increased amount in the blood may be a sign of tissue damage and some types of cancer or other diseases. Also called lactate dehydrogenase and lactic acid dehydrogenase.

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

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

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

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 bronchi, and lymph nodes.

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.

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.

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

A tiny organ in the cerebrum that produces melatonin. Also called pineal body and pineal organ.

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

To come back or to return.

Something that may increase the chance of developing a disease. Some examples of risk factors for cancer include age, a family history of certain cancers, use of tobacco products, certain eating habits, obesity, lack of exercise, exposure to radiation or other cancer-causing agents, and certain genetic changes.

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.

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

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.

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 cancerous), or malignant (cancerous). 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 (ultrasound) 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 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.