Types of Cancer   /   Pediatric Cancers   /   Brain Tumors (Childhood)   /   NCI Resources

NCI/PDQ^® Patients: Childhood Extracranial Germ Cell Tumors Treatment (PDQ®)

National Cancer Institute
Last Modified: December 7, 2012

TABLE OF CONTENTS

• General Information About Childhood Extracranial Germ Cell Tumors

• Stages of Childhood Extracranial Germ Cell Tumors

• Recurrent Childhood Extracranial Germ Cell Tumors

• Treatment Option Overview

• Treatment Options for Childhood Extracranial Germ Cell Tumors
  • Mature and Immature Teratomas
  • Malignant Gonadal Germ Cell Tumors
    • Malignant Testicular Germ Cell Tumors
    • Malignant Ovarian Germ Cell Tumors
  • Malignant Extragonadal Germ Cell Tumors
  • Recurrent Childhood Malignant Extracranial Germ Cell Tumors

• To Learn More About Childhood Cancer

• Changes to This Summary (12/07/2012)

• Get More Information From NCI

• About PDQ®

General Information About Childhood Extracranial Germ Cell Tumors

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. Immature teratomas are not cancer. They often contain several different types of tissue such as hair, muscle, and bone.

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 (-hCG).
• Choriocarcinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (-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, seminoma and nonseminoma. (See the PDQ® summary on Testicular Cancer Treatment for more information.)

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

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 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 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. Talk with your child's doctor if you think your child may be at risk. 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. Check with a doctor if your child has any of the following problems:

• 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 (-hCG).

• 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.

Stages of Childhood Extracranial Germ Cell Tumors

After a childhood extracranial germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread from where the tumor started to nearby areas or to other parts of the body.

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:

• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging.
• Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
• Thoracentesis: The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.
• Paracentesis: The removal of fluid from the space between the lining of the abdomen and the organs in the abdomen, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.

The results from tests and procedures used to detect and diagnose childhood extracranial germ cell tumor may also be used in staging.

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 stages are commonly used for childhood nonseminoma testicular germ cell tumors:

Stage I

In stage I, the cancer is found only in the testicle and is completely removed by surgery. Tumor marker levels return to normal after surgery.

Stage II

In stage II, the cancer is removed by surgery and some cancer cells remain in the scrotum or cancer that can only be seen with a microscope has spread to the scrotum or spermatic cord. Tumor marker levels do not return to normal after surgery and may increase.

Stage III

In stage III, the cancer has spread to one or more lymph nodes in the abdomen and is not completely removed by surgery. The cancer that remains after surgery can be seen without a microscope.

Stage IV

In stage IV, the cancer has spread to distant parts of the body such as the liver.

The following stages may be used for childhood ovarian germ cell tumors:

Stage I

In stage I, the cancer is in the ovary and can be completely removed by surgery.

Stage II

In stage II, one of the following is true:

• The cancer is not completely removed by surgery. The remaining cancer can be seen with a microscope only.
• The cancer has spread to the lymph nodes and can be seen with a microscope only.
• The cancer has spread to the capsule (outer covering) of the ovary.

Stage III

In stage III, one of the following is true:

• The cancer is not completely removed by surgery. The remaining cancer can be seen without a microscope.
• The cancer has spread to lymph nodes and the lymph nodes are 2 centimeters or larger.
• The cancer is found in fluid in the abdomen.

Stage IV

In stage IV, the cancer has spread to the lung, liver, brain, or bone.

Another staging system which may be used for childhood ovarian germ cell tumors is as follows:

Stage I

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.

• Stage IA: Cancer is found in a single ovary.
• Stage IB: Cancer is found in both ovaries.
• Stage IC: Cancer is found in one or both ovaries and one of the following is true:
  • cancer is found on the outside surface of one or both ovaries; or
  • the capsule (outer covering) of the tumor has ruptured (broken open); or
  • cancer cells are found in fluid that has collected in the abdomen; or
  • cancer cells are found in washings of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen).

Stage II

In stage II, cancer is found in one or both ovaries and has spread into other areas of the pelvis. Stage II is divided into stage IIA, stage IIB, and stage IIC.

• Stage IIA: Cancer has spread to the uterus and/or the fallopian tubes (the long slender tubes through which eggs pass from the ovaries to the uterus).
• Stage IIB: Cancer has spread to other tissue within the pelvis such as the bladder, rectum, or vagina.
• Stage IIC: Cancer has spread to the uterus and/or fallopian tubes and/or other tissue within the pelvis and one of the following is true:
  • cancer is found on the outside surface of one or both ovaries; or
  • the capsule (outer covering) of the tumor has ruptured (broken open); or
  • cancer cells are found in fluid that has collected in the abdomen; or
  • cancer cells are found in washings of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen).

Stage III

In stage III, cancer is found in one or both ovaries and has spread to other parts of the abdomen. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC as follows:

• Stage IIIA: The tumor is found only in the pelvis, but cancer cells that only can be seen with a microscope have spread to the surface of the peritoneum (tissue that lines the abdominal wall and covers most of the organs in the abdomen) or to the small bowel.
• Stage IIIB: Cancer has spread to the peritoneum but is 2 centimeters or smaller in diameter.
• Stage IIIC: Cancer has spread to the peritoneum and is larger than 2 centimeters in diameter and/or has spread to lymph nodes in the abdomen.

Cancer that has spread to the surface of the liver is also considered stage III disease.

Stage IV

In stage IV, cancer is found in one or both ovaries and has metastasized (spread) beyond the abdomen to other parts of the body.

Cancer that has spread to tissues in the liver is also considered stage IV disease.

The following stages are commonly used for extragonadal extracranial germ cell tumors:

Stage I

In stage I, the cancer is in one place and can be completely removed by surgery. For tumors at the base of the tailbone, the cancer and tailbone are completed removed by surgery. Tumor marker levels return to normal after surgery.

Stage II

In stage II, the cancer has spread to nearby tissues and/or lymph nodes and is not completely removed by surgery. The cancer remaining after surgery can be seen with a microscope only. Tumor marker levels do not return to normal after surgery and may increase.

Stage III

In stage III, one of the following is true:

• The cancer is not completely removed by surgery. The cancer remaining after surgery can be seen without a microscope.
• The cancer has spread to lymph nodes and is larger than 2 centimeters in diameter.

Stage IV

In stage IV, the cancer has spread to distant parts of the body, including the liver.

Recurrent Childhood Extracranial Germ Cell Tumors

Recurrent childhood extracranial germ cell tumor is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body.

The number of patients who have tumors that come back is small. Most recurrent germ cell tumors occur within three years of surgery. About half of the teratomas that recur in the sacrum or coccyx are malignant, so follow-up is important.

Treatment Option Overview

There are different types of treatment for children with extracranial germ cell tumors.

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.

Children with extracranial germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

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:

• Pediatric surgeon.
• Pediatric hematologist.
• Radiation oncologist.
• Endocrinologist.
• Pediatric nurse specialist.
• Rehabilitation specialist.
• Psychologist.
• Social worker.
• Geneticist.

Some cancer treatments cause side effects months or years after treatment has ended.

Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:

• Physical problems.
• Changes in mood, feelings, thinking, learning, or memory.
• Second cancers (new types of cancer).

For example, late effects of surgery to remove tumors in the sacrum or coccyx include constipation, loss of bowel and bladder control, and scars.

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. (See the PDQ® summary on Late Effects of Treatment for Childhood Cancer for more information).

Three types of standard treatment are used:

Surgery

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. The following types of surgery may be used:

• Resection: Surgery to remove tissue or part or all of an organ. If cancer is in the coccyx, the entire coccyx is removed.
• Tumor debulking: A surgical procedure in which as much of the tumor as possible is removed. Some tumors may not be able to be completely removed.
• Radical inguinal orchiectomy: Surgery to remove one or both testicles through an incision (cut) in the groin.
• Unilateral salpingo-oophorectomy: Surgery to remove one ovary and one fallopian tube.

Watchful waiting

Watchful waiting is closely monitoring a patient's condition without giving any treatment until symptoms appear or change. For childhood extracranial germ cell tumors, this includes physical exams, imaging tests, and tumor marker tests.

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 into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI Web site.

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 listing of clinical trials.

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

For childhood extracranial germ cell tumors, alpha-fetoprotein (AFP) tests are done to see if treatment is working. Continued high levels of AFP may mean the cancer is still growing. For at least 3 years after surgery, follow-up will include regular physical exams, imaging tests, and tumor marker tests.

Treatment Options for Childhood Extracranial 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 child's doctor for clinical trials that are not listed here but may be right for your child.

Mature and Immature Teratomas

Treatment of mature and immature teratomas in the sacrum or coccyx is usually surgery followed by watchful waiting. Most teratomas can be removed completely. If the tumor is in the coccyx, the entire coccyx is removed. Chemotherapy may be given if the tumor comes back.

Treatment of mature and immature teratomas that are not in the sacrum or coccyx is usually surgery followed by watchful waiting. A second surgery may be done to remove any remaining cancer.

Sometimes a mature or immature teratoma also has malignant cells. The teratoma and malignant cells may need to be treated differently. The best treatment for the malignant cells is not known.

Regular follow-up exams with imaging tests and the alpha-fetoprotein (AFP) tumor marker test will be done for at least 3 years.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood teratoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Malignant Gonadal Germ Cell Tumors

Malignant Testicular Germ Cell Tumors

Treatment of malignant testicular germ cell tumors may include the following:

For boys younger than 15 years:

• Surgery (radical inguinal orchiectomy) followed by watchful waiting for stage I tumors. Chemotherapy may be given if the tumor comes back.
• Surgery (radical inguinal orchiectomy) followed by combination chemotherapy for stage II-IV tumors.
• A clinical trial of fewer cycles of chemotherapy after surgery.

For boys 15 years and older:

Malignant testicular germ cell tumors in boys 15 years and older are treated differently than they are in young boys. Surgery may include removal of lymph nodes in the abdomen. Treatment depends on whether the tumor is a seminoma or a nonseminoma. (See the PDQ® summary on Testicular Cancer Treatment for more information.)

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood malignant testicular germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Malignant Ovarian Germ Cell Tumors

Treatment of childhood malignant ovarian germ cell tumors in young girls may include the following:

• Surgery (unilateral salpingo-oophorectomy<