Childhood Extracranial Germ Cell Tumors Treatment (PDQ®)

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General Information About Childhood Extracranial Germ Cell Tumors

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.

Extracranial germ cell tumors may be benign (noncancer) or malignant (cancer).

Extracranial germ cell tumors are grouped into mature teratomas, immature teratomas, or malignant germ cell tumors:

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

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

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 (ß-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 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:

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.

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.

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:

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 (ß-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.

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

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.

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.

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.

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.

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.

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

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

In stage II, the cancer:

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

In stage III, the cancer:

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

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).

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.

Pea, peanut, walnut, and lime show tumor sizes.

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:

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

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.

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.

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.

In stage III, the cancer:

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

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.

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:

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).

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:

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 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.

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

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.

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.

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.

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 doctor for clinical trials that are not listed here but may be right for you.

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.

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:

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:

The treatment for adolescents and young adults with ovarian germ cell tumor is similar to the treatment for adults. (See the PDQ treatment summary on Ovarian Germ Cell Tumors 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 ovarian 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 Extragonadal Germ Cell Tumors

Treatment of childhood extragonadal malignant germ cell tumors may include the following:

(See the PDQ treatment summary on Extragonadal Germ Cell Tumors for more information.)

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood extragonadal 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.

Recurrent Childhood Malignant Extracranial Germ Cell Tumors

There is no standard treatment for recurrent childhood malignant extracranial germ cell tumors. Treatment is usually within in a clinical trial and may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood malignant 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.

To Learn More About Childhood Cancer

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



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