Childhood Extracranial Germ Cell Tumors Treatment (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
Childhood extracranial germ cell tumors may be benign or malignant.
Extracranial germ cell tumors may be
There are three types of extracranial germ cell tumors.
Extracranial germ cell tumors are grouped into
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
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 ahormone calledalpha-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 Germ Cell Tumors
Gonadal germ cell tumors form in the testicles or ovaries.
Testicular Germ Cell Tumors
Testicular germ cell tumors in teenagers and young adults are different from those that form in early childhood. They are more like
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
Ovarian Germ Cell Tumors
Extragonadal Extracranial Germ Cell Tumors
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 lowerspine that forms part of thepelvis ).Coccyx (the small bone at the bottom of the spine, also called thetailbone ).Mediastinum (the area between thelungs ).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
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
Having certain
genetic syndromes may increase the risk of developing childhood germ cell tumors:Klinefelter syndrome may increase the risk of developing germ cell tumors in the mediastinum.Swyer syndrome may increase the risk of developing germ cell tumors in the testes or ovaries.
Having an
undescended testicle may increase the risk of developing a testicular germ cell tumor.
Signs of childhood extracranial germ cell tumors depend on the type of tumor and where it is in the body.
Different tumors may cause the following signs and symptoms. Other conditions may cause these same symptoms. A doctor should be consulted if any of these problems occur.
Most tumors of the sacrum and coccyx can be seen as a lump.
A testicular tumor may cause a painless lump in the testicles.
An ovarian germ cell tumor may cause:
Pain or a lump in the abdomen.
Fever .Constipation .No
menstruation .Unusual
vaginal bleeding.
Imaging studies and blood tests are used to detect (find) and diagnose childhood extracranial germ cell tumors.
The following tests and procedures may be used:
Physical exam andhistory : 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 ofblood is checked to measure the amounts of certain substances released into the blood byorgans ,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 calledtumor 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).
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 : Alaboratory test in which cells in a sample of tissue are viewed under amicroscope to look for certain changes in thechromosomes .Immunohistochemistry study: A laboratory test in which a substance such as anantibody ,dye , orradioisotope is added to a sample of cancer tissue to test for certainantigens . This type of study is used to tell the difference between different types of cancer.Chest x-ray : Anx-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 beinjected into avein 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 asonogram . 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 apathologist to check for signs of cancer. In some cases, the tumor is removed duringsurgery and then a biopsy is done.
Certain factors affect prognosis (chance of recovery) and treatment options.
The
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 hasrecurred (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
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 dividingcells , such as cancer cells, in the bone. A very small amount ofradioactive material isinjected into avein and travels through the bloodstream. The radioactive material collects in the bones and is detected by ascanner .Thoracentesis : The removal offluid from the space between the lining of the chest and thelung , using a needle. Apathologist views the fluid under amicroscope to look for cancer cells.Paracentesis : The removal of fluid from the space between the lining of theabdomen and theorgans 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
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
Through
tissue . Cancerinvades the surrounding normal tissue.Through the
lymph system . Cancer invades the lymph system and travels through thelymph vessels to other places in the body.Through the
blood . Cancer invades theveins andcapillaries and travels through the blood to other places in the body.
When cancer
The following stages are commonly used for childhood nonseminoma testicular germ cell tumors:
Stage I
In stage I, the
Stage II
In stage II, the
Stage III
In stage III, the
Stage IV
In stage IV, the
The following stages may be used for childhood ovarian germ cell tumors:
Stage I
In stage I, the
Stage II
In stage II, one of the following is true:
The
cancer is not completely removed bysurgery . The remaining cancer can be seen with amicroscope 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 theovary .
Stage III
In stage III, one of the following is true:
The
cancer is not completely removed bysurgery . The remaining cancer can be seen without amicroscope .The cancer has spread to
lymph nodes and the lymph nodes are 2centimeters or larger.The cancer is found in
fluid in theabdomen .
Stage IV
In stage IV, the
Another staging system which may be used for childhood ovarian germ cell tumors is as follows:
Stage I
In stage I,
Stage IA:
Cancer is found in a singleovary .Stage IB:
Cancer is found in bothovaries .Stage IC:
Cancer is found in one or bothovaries 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 thetumor has ruptured (broken open); orcancer
cells are found influid that has collected in theabdomen ; orcancer cells are found in
washings of theperitoneal cavity (the bodycavity that contains most of theorgans in the abdomen).
Stage II
In stage II,
Stage IIA:
Cancer has spread to theuterus and/or thefallopian tubes (the long slender tubes through which eggs pass from theovaries to theuterus ).Stage IIB:
Cancer has spread to othertissue within thepelvis such as thebladder ,rectum , orvagina .Stage IIC:
Cancer has spread to theuterus and/orfallopian tubes and/or othertissue within thepelvis and one of the following is true:cancer is found on the outside surface of one or both
ovaries ; orthe
capsule (outer covering) of thetumor has ruptured (broken open); orcancer
cells are found influid that has collected in theabdomen ; orcancer cells are found in
washings of theperitoneal cavity (the body cavity that contains most of theorgans in the abdomen).
Stage III
In stage III,
Stage IIIA: The
tumor is found only in the pelvis, but cancercells that only can be seen with amicroscope have spread to the surface of theperitoneum (tissue that lines theabdominal wall and covers most of the organs in theabdomen ) or to the smallbowel .Stage IIIB: Cancer has spread to the
peritoneum but is 2centimeters or smaller indiameter .Stage IIIC: Cancer has spread to the
peritoneum and is larger than 2 centimeters in diameter and/or has spread to lymph nodes in theabdomen .
Cancer that has spread to the surface of the
Stage IV
In stage IV,
Cancer that has spread to
The following stages are commonly used for extragonadal extracranial germ cell tumors:
Stage I
In stage I, the
Stage II
In stage II, the
Stage III
In stage III, one of the following is true:
The
cancer is not completely removed bysurgery . The cancer remaining after surgery can be seen without amicroscope .The cancer has spread to
lymph nodes and is larger than 2centimeters in diameter.
Stage IV
In stage IV, the
Recurrent Childhood Extracranial Germ Cell Tumors
The number of patients who have
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
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 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.
Physical problems.
Changes in mood, feelings, thinking, learning, or memory.
Second cancers (new types of cancer).
For example, late effects of
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
Three types of standard treatment are used:
Surgery
Surgery to completely remove the tumor is done whenever possible. If the tumor is very large,
Resection : Surgery to remove tissue or part or all of anorgan . 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 bothtesticles through anincision (cut) in thegroin .Unilateral salpingo-oophorectomy : Surgery to remove oneovary and onefallopian tube .
Watchful waiting
Chemotherapy
Chemotherapy is a cancer treatment that uses
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
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
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
Follow-up tests may be needed.
Some of the tests that were done to
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
For childhood extracranial germ cell tumors,
Treatment Options for Childhood Extracranial Germ Cell Tumors
A link to a list of current
Mature and Immature Teratomas
Treatment of
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
Sometimes a mature or immature teratoma also has
Regular
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
For boys younger than 15 years:
Surgery (radicalinguinal orchiectomy ) followed bywatchful waiting for stage Itumors .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 lowerdoses and 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
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
Surgery (unilateral salpingo-oophorectomy ) followed bywatchful waiting for stage Idysgerminoma orimmature teratoma tumors .Chemotherapy may be given if the tumor comes back.Surgery (unilateral salpingo-oophorectomy ) followed bycombination chemotherapy for stages II-IVtumors .Chemotherapy to make the tumor smaller, followed by surgery (unilateral salpingo-oophorectomy).
Surgery (
tumor debulking ) followed by chemotherapy.A
clinical trial of lowerdoses and fewer cycles of chemotherapy after surgery.A clinical trial of surgery (unilateral salpingo-oophorectomy) followed by
watchful waiting for stage I tumors that are not a dysgerminoma or immature teratoma.
The treatment for adolescents and young adults with ovarian germ cell tumor is similar to the treatment for adults. (See the
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
Surgery to removetumors in themediastinum (the area between thelungs ).Surgery followed by
combination chemotherapy .Combination chemotherapy followed by surgery.
Chemotherapy only.A
clinical trial of lowerdoses and fewer cycles of chemotherapy.A clinical trial of a new chemotherapy
regimen .
(See the
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
Surgery followed bycombination chemotherapy , for malignanttesticular germ cell tumors thatrecur after being treated with surgery andwatchful waiting .Surgery for
tumors that come back in thesacrum orcoccyx .Chemotherapy may be given before surgery, to shrink the tumor. If any of the tumor remains after surgery,radiation therapy may be added.Surgery followed by chemotherapy, for malignant
ovarian germ cell tumors that recur after being treated with surgery and watchful waiting.
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 information from the
For more childhood
What You Need to Know About? Cancer
Childhood Cancers
CureSearch for Children's Cancer
Late Effects of Treatment for Childhood Cancer
Adolescents and Young Adults with Cancer
Young People with Cancer: A Handbook for Parents
Care for Children and Adolescents with Cancer
Understanding Cancer Series: Cancer
Cancer Staging
Coping with Cancer: Supportive and Palliative Care
Cancer Library
Information for Survivors/Caregivers/Advocates
Get More Information From NCI
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.
Chat online
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:
NCI Public Inquiries Office
Suite 3036A
6116 Executive Boulevard, MSC8322
Bethesda, MD 20892-8322
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).
Changes to This Summary (12/08/2011)
The
Changes were made to this summary to match those made to the health professional version.
About PDQ
PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
PDQ contains cancer information summaries.
The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.
The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.
Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.
PDQ also contains information on clinical trials.
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." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

