Childhood Extracranial Germ Cell Tumors Treatment (PDQ®)

Childhood extracranial germ cell tumors form from germ cells in parts of the body other than the brain.

A germ cell is a type of cell that forms as a fetus (unborn baby) develops. These cells later become sperm in the testicles or eggs in the ovaries. Sometimes while the fetus is forming, germ cells travel to parts of the body where they should not be and grow into a germ cell tumor. The tumor may form before or after birth.

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 of age.

See the PDQ summary on Childhood Central Nervous System Germ Cell Tumors Treatment for information on intracranial (inside the brain) germ cell tumors.

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, and malignant germ cell tumors:

Mature Teratomas

Mature teratomas are the most common type of extracranial germ cell tumor. Mature teratomas are benign tumors and not likely to become cancer. They usually occur in the sacrum or coccyx (bottom part of the spine) in newborns or in the ovaries of girls at the start of puberty. The cells of mature teratomas look almost like normal cells under a microscope. Some mature teratomas release enzymes or hormones that cause signs and symptoms of disease.

Immature Teratomas

Immature teratomas also usually occur in the sacrum or coccyx (bottom part of the spine) in newborns or the ovaries of girls at the start of puberty. Immature teratomas have cells that look very different from normal cells under a microscope. Immature teratomas may be cancer. They often have several different types of tissue in them, such as hair, muscle, and bone. Some immature teratomas release enzymes or hormones that cause signs and symptoms of disease.

Malignant Germ Cell Tumors

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

  • Germinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (β-hCG). There are three types of germinomas.
    • Dysgerminomas form in the ovary in girls.
    • Seminomas form in the testicle in boys.
    • Germinomas form in areas of the body that are not the ovary or testicle.
  • Nongerminomas: There are four types of nongerminomas.
    • Yolk sac tumors make a hormone called alpha-fetoprotein (AFP). They can form in the ovary, testicle, or other areas of the body.
    • Choriocarcinomas make a hormone called beta-human chorionic gonadotropin (β-hCG). They can form in the ovary, testicle, or other areas of the body.
    • Embryonal carcinomas may make a hormone called β-hCG and/or a hormone called AFP. They can form in the testicle or other parts of the body, but not in the ovary.
    • Mixed germ cell tumors are made up of both malignant germ cell tumor and teratoma. They can form in the ovary, testicle, or other areas of the body.
Childhood extracranial germ cell tumors are grouped as gonadal or extragonadal.

Malignant extracranial germ cell tumors are gonadal or extragonadal.

Gonadal Germ Cell Tumors

Gonadal germ cell tumors form in the testicles in boys or ovaries in girls.

Testicular Germ Cell Tumors

Testicular germ cell tumors are divided into two main types, seminoma and nonseminoma.

  • Seminomas make a hormone called beta-human chorionic gonadotropin (β-hCG).
  • Nonseminomas are usually large and cause signs or symptoms. They tend to grow and spread more quickly than seminomas.

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.

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

Ovarian Germ Cell Tumors

Ovarian germ cell tumors are more common in teenage girls and young women. Most ovarian germ cell tumors are benign teratomas. Sometimes immature teratomas, dysgerminomas, yolk sac tumors, and mixed germ cell tumors (cancer) occur. (See the PDQ summary on Ovarian Germ Cell Tumors Treatment for more information.)

Extragonadal Extracranial Germ Cell Tumors

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

Most extragonadal extracranial germ cell tumors 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 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:
    • Klinefelter syndrome may increase the risk of germ cell tumors in the mediastinum.
    • Swyer syndrome may increase the risk of germ cell tumors in the testicles or ovaries.
    • Turner syndrome may increase the risk of germ cell tumors in the ovaries.
  • Having an undescended testicle may increase the risk of developing a testicular germ cell tumors.
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 signs and symptoms. Check with a doctor if your child has any of the following:

  • A lump in the abdomen or lower back.
  • A painless lump in the testicle.
  • Pain in the abdomen.
  • Fever.
  • Constipation.
  • In females, no menstrual periods.
  • In females, 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.
  • 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.
  • 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 (NMRI).
  • 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 following tests may be done on the sample of tissue that is removed:

    • 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: A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.
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.

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, such as the lymph nodes. This procedure is also called nuclear magnetic resonance imaging.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or lymph nodes, 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.
  • 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 with cancer 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 tumors may also be used in staging.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if an extracranial germ cell tumor spreads to the liver, the cancer cells in the liver are actually cancerous germ cells. The disease is metastatic extracranial germ cell tumor, not liver cancer.

The following stages are commonly used for childhood nonseminoma testicular germ cell tumors:
Stage I

In stage I, the cancer is found in the testicle only and is completely removed by surgery.

Stage II

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

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 and the capsule (outer covering) of the ovary has not ruptured (broken open).

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. Cancer in the lymph nodes can be seen without a microscope.
  • 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 one 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:

  • Stage IIIA: The tumor is found only in the pelvis only, but cancer cells that can only 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 and 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 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 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 in the sacrum or coccyx (bottom part of the spine), the sacrum and coccyx are completely removed by surgery. Tumor marker levels return to normal after surgery.

Stage II

In stage II, the cancer has spread to the capsule (outer covering) and/or lymph nodes. The cancer is not completely removed by surgery and the cancer remaining after surgery can be seen with a microscope only. Tumor marker levels do not return to normal after surgery or 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, brain, bone, or lung.

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 come back within three years of surgery. About half of the teratomas that recur in the sacrum or coccyx are malignant, so follow-up is important.

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:

  • Pediatrician.
  • Pediatric surgeon.
  • Pediatric hematologist.
  • Radiation oncologist.
  • Endocrinologist.
  • Pediatric nurse specialist.
  • Rehabilitation specialist.
  • Child life professional.
  • 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.
  • 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 on the same side.
Observation

Observation is closely monitoring a patient’s condition without giving any treatment until signs or 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.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.

High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

Hyperthermia therapy

Hyperthermia therapy is a treatment in which body tissue is heated above normal temperature to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation and certain anticancer drugs.

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 and beta-human chorionic gonadotropin (β-hCG) tests are done to see if treatment is working. Continued high levels of AFP or β-hCG 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 of mature teratomas that are not in the sacrum or coccyx (bottom part of the spine) includes the following:

  • Surgery to remove the tumor followed by observation.

Treatment of immature teratomas that are not in the sacrum or coccyx includes the following:

  • Surgery to remove the tumor followed by observation for stage I tumors.
  • Surgery to remove the tumor and combination chemotherapy for stage II–IV tumors. It is not known if chemotherapy will help the patient live longer.

Treatment of immature teratomas that are in the sacrum or coccyx includes the following:

  • Surgery (removal of the sacrum and coccyx) followed by observation.

Sometimes a mature or immature teratoma also has malignant cells. The teratoma and malignant cells may need to be treated differently.

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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.

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

For boys younger than 15 years:

  • Surgery (radical inguinal orchiectomy) followed by observation for stage I tumors.
  • Surgery (radical inguinal orchiectomy) followed by combination chemotherapy for stage II-IV tumors. A second surgery may be done to remove any tumor remaining after chemotherapy.

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. (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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.

Treatment of stage I dysgerminomas in young girls may include the following:

  • Surgery (unilateral salpingo-oophorectomy) followed by observation. Combination chemotherapy may be given if the tumor comes back.

Treatment of stages II–IV dysgerminomas in young girls may include the following:

  • Surgery (unilateral salpingo-oophorectomy) followed by combination chemotherapy.
  • Combination chemotherapy to shrink the tumor, followed by surgery (unilateral salpingo-oophorectomy).

Treatment of stage I nongerminomas in young girls may include the following:

  • Surgery followed by observation.
  • Surgery followed by combination chemotherapy.

Treatment of stages II–IV nongerminomas in young girls may include the following:

  • Surgery followed by combination chemotherapy. A second surgery may be done to remove any remaining cancer.
  • Biopsy followed by combination chemotherapy to shrink the tumor and sometimes surgery for tumors that cannot be removed by surgery when cancer is diagnosed.

The treatment for adolescents and young adults with ovarian germ cell tumor is much like 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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.

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

  • Combination chemotherapy to shrink the tumor followed by surgery to remove the sacrum and coccyx (bottom part of the spine) for tumors that are in the sacrum or coccyx.
  • Combination chemotherapy to shrink the tumor followed by surgery to remove tumors that are in the mediastinum.
  • Biopsy followed by combination chemotherapy to shrink the tumor and surgery to remove tumors that are in the abdomen.
  • Surgery to remove the tumor followed by combination chemotherapy for tumors of the head and neck.

Treatment of malignant extragonadal extracranial germ cell tumors in places not already described includes the following:

  • Surgery followed by combination chemotherapy.

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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.

There is no standard treatment for recurrent childhood malignant extracranial germ cell tumors. Treatment depends on the following:

  • The type of treatment given when the cancer was diagnosed.
  • How the tumor responded to the initial treatment.

Treatment is usually within a clinical trial and may include the following:

  • Surgery followed by combination chemotherapy, for most malignant extracranial germ cell tumors including immature teratomas, malignant testicular germ cell tumors, and malignant ovarian germ cell tumors.
  • Surgery for tumors that come back in the sacrum or coccyx (bottom part of the spine), if surgery to remove the sacrum and coccyx was not done when the cancer was diagnosed. Chemotherapy may be given before surgery, to shrink the tumor. If any tumor remains after surgery, radiation therapy may also be given.
  • Combination chemotherapy for stage I malignant testicular germ cell tumors and stage I ovarian dysgerminomas.
  • High-dose chemotherapy and stem cell transplant.
  • Radiation therapy followed by surgery to remove the tumor in the brain for cancer that has spread to the brain.

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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI Web site.

For more information from the National Cancer Institute about childhood extracranial germ cell tumors, see the following.

For more childhood cancer information and other general cancer resources, see the following:

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Changes were made to this summary to match those made to the health professional version.

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This PDQ cancer information summary has current information about the treatment of childhood extracranial germ cell tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials are listed in PDQ and can be found online at NCI's Web site. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”

The best way to cite this PDQ summary is:

National Cancer Institute: PDQ® Childhood Extracranial Germ Cell Tumors Treatment. Bethesda, MD: National Cancer Institute. Date last modified <MM/DD/YYYY>. Available at: http://cancer.gov/cancertopics/pdq/treatment/extracranial-germ-cell/Patient. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 2,000 scientific images.

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Coping with Cancer: Financial, Insurance, and Legal Information page.

More information about contacting us or receiving help with the Cancer.gov Web site can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the Web site’s Contact Form.

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.

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The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 8:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write to us

For more information from the NCI, please write to this address:

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  • Room 2E532 MSC 9760
  • Bethesda, MD 20892-9760

Search the NCI Web site

The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).

2014-11-21