Childhood Extracranial Germ Cell Tumors
Description
What is childhood extracranial germ cell tumor?
Germ cells are reproductive cells that develop into testicles in males and ovaries in females. Sometimes these cells travel to other areas of the body, such as the chest, abdomen, or brain, and may turn into a rare type of cancer called germ cell tumor. This summary covers germ cell tumors that occur extracranially (everywhere but in the brain).
Extracranial germ cell tumors can be benign (not cancerous) or malignant (cancerous). Most germ cell tumors are benign and are very rare in children younger than age 15. Germ cell tumors of early childhood have biological characteristics which are different than those that occur in adolescents and young adults. The location of the tumor and the age of the child make a difference in the prognosis (chance of recovery) and in how the tumor is treated. The major types of germ cell tumors by location and age are:
Germ cell tumors of the fetal/ neonatal period
Most germ cell tumors that are present at birth are not cancerous. These tumors are usually located outside the testicles in areas such as the tailbone, chest and neck. Fetal/ neonatal germ cell tumors can cause complications such as premature birth.
Testicular germ cell tumors of early childhood
This type of germ cell tumor forms within the testis of young boys. The treatment for this type of germ cell tumor is covered later in this summary.
Testicular germ cell tumors of adolescence and young adulthood
This type of germ cell tumor forms within the testes of older boys. Testicular germ cell tumors are classified as either seminoma or nonseminoma. This classification is important for planning treatment because seminomas are more sensitive to radiation therapy. (Refer to the PDQ summary on Testicular Cancer for more information.)
Extragonadal, extracranial germ cell tumors of early childhood
This includes any type of germ cell tumor that is not located in the reproductive organs (testicles or ovaries) or in the brain. These germ cell tumors are usually located in the sacrum (a triangular-shaped section of fused bone located between the hip bones at the base of the spine) and the coccyx (the fused bones located on the end of the sacrum; also called the tailbone). The treatment for this type of germ cell tumor is covered later in this summary.
Extragonadal, extracranial germ cell tumors of adolescence and young adulthood
This type of germ cell tumor is usually located within the chest. The treatment for this type of germ cell tumor is covered later in this summary.
Ovarian germ cell tumors
Ovarian germ cell tumor, a rare type of cancer that affects teenage girls and young women, is a disease in which cancer (malignant) cells are found in egg-making cells in an ovary. An ovary is a small organ that holds the eggs that can develop into a baby. There are 2 ovaries: one located on the left side of the uterus (the hollow, pear-shaped organ where a baby grows) and one located on the right. The treatment for this type of germ cell tumor is covered later in this summary (refer to the PDQ summary on Ovarian Germ Cell Tumors for more information).
Germ cell tumors form in developing cells and usually contain tissues that are foreign to the location of the tumor. Germ cell tumors can further be classified as teratomas or malignant germ cell tumors. Teratomas can be either mature (well differentiated tissue that forms a tumor that is less likely to become cancer) or immature (undifferentiated tissue that can spread and become cancer). Most teratomas are mature and develop into benign tumors.
Stage Explanation
Once a germ cell tumor is found (diagnosed), tests will be done to find out if the germ cell tumor is benign or malignant. If the tumor is malignant, more tests will be done to find out if the cancercells have spread to other parts of the body (staging). In order to stage a germ cell tumor, the patient may undergo a surgical procedure. Knowing the stage of the disease will assist the doctor in effectively planning further treatment.
Extracranial Germ Cell Tumors
The following stages are used for all extracranial germ cell tumors:
Stage I
Cancer has not spread from the tumor to surrounding tissues or lymph nodes and can be surgically removed with no cancer cells remaining.
Stage II
Cancer has spread to surrounding tissues or lymph nodes, and surgery cannot remove all cancer cells from the surrounding tissues.
Stage III
Cancer has spread to surrounding tissues, has affected several lymph nodes, is found in fluid in the abdomen, and surgery cannot remove the entire tumor from the surrounding tissues.
Stage IV
Cancer has spread to other organs in the body.
Recurrent
Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the original site of the tumor or in another place.
Ovarian Germ Cell Tumor
The following stages are used for ovarian germ cell tumor:
Stage I
Cancer is found in either one or both of the ovaries; it has not spread to the surrounding tissue. Tumor cells may be present in peritoneal (abdominal) fluid.
Stage II
Cancer is found in one or both ovaries and has spread to the uterus, and/or the fallopian tubes (the pathway used by egg cells moving from the ovary to the uterus), and/or other body parts within the pelvis (bladder, rectum,vagina). Tumor cells may be present in abdominalfluid.
Stage III
Cancer is found in one or both ovaries and has spread to lymph nodes or to other body parts inside the abdomen (outside of the pelvis), such as the surface of the liver or intestine.
Stage IV
Cancer is found in one or both ovaries and has spread outside the abdomen or has spread to the inside of the liver.
Recurrent
Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the ovary or in another place.
Treatment Option Overview
Experienced doctors working together may provide the best treatment for children with extracranial germ cell tumors. Your child’s treatment will often be coordinated by a pediatriconcologist, a doctor who specializes in cancer in children. The pediatric oncologist may refer your child to other specialists, such as a pediatric surgeon, a psychologist, a radiation oncologist, and other doctors who specialize in the type of treatment your child requires.
Treatment for extracranial germ cell tumor depends upon the location of the tumor, the stage of the tumor, and the type of tumor. The types of treatment used for extracranial germ cell tumor are:
More than one method of treatment may be used, depending on the type of extracranial germ cell tumor and how much cancer the patient has in his or her body. Complete or near complete surgical removal of the tumor is often possible. If the tumor cannot be completely removed, chemotherapy may also be given.
Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy drugs may be taken by mouth or injected into a vein (intravenous) or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body.
Your child may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or you may choose to have your child enter a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to test new treatments and to find better ways to treat people with cancer. For more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615
Childhood Mature and Immature Teratomas
Treatment will depend on whether the tumor is a mature or immature teratoma. If the tumor is a mature teratoma, the treatment will be surgery to remove the tumor and possibly some of the surrounding tissues or structures. If the tumor is an immature teratoma, treatment will be surgery with or without chemotherapy.
Childhood Malignant Testicular Germ Cell Tumor
Treatment will depend on the age of the child. The majority of childhood malignanttesticulargerm cell tumors occur in boys younger than 4 years of age. Surgery is the most common form of treatment for testicular germ cell tumor. A doctor may take out the tumor by removing one or both testicles through an incision (cut) in the groin. This is called a radical inguinal orchiectomy. Treatment for boys younger than 5 years of age will be radical inguinal orchiectomy with or without chemotherapy.
The treatment for adolescents and young adults with testicular germ cell tumor is the same as the treatment for adults. (Refer to the PDQ summary on Testicular Cancer for more information.)
Childhood Malignant Ovarian Germ Cell Tumor
Treatment will depend on the age of the child and the specific tumor type. Surgery is the most common form of treatment for ovarian germ cell tumor. A doctor may take out the cancer using one of the following operations:
Treatment for young girls with early stage ovarian germ cell tumor will be unilateral salpingo-oophorectomy. Treatment for young girls with advanced stage ovarian germ cell tumor will be unilateral salpingo-oophorectomy with or without chemotherapy.
The treatment for adolescents and young adults with ovarian germ cell tumor is similar to the treatment for adults. (Refer to the PDQ summary on Ovarian Germ Cell Tumors for more information.)
Childhood Extragonadal Malignant Germ Cell Tumor
Treatment for childhood extragonadal malignant germ cell tumor depends on the size and location of the tumor. Treatment for smaller tumors will be surgery to remove the tumor followed by chemotherapy. Treatment for larger tumors will be a biopsy (a surgical procedure to remove a small portion of the tumor) followed by chemotherapy to reduce the size of the tumor, possibly followed by surgery to remove any remaining tumor.
Recurrent Childhood Malignant Germ Cell Tumor
Due to the small number of childhood extracranial germ cell tumors and the effectiveness of treatment, the number of patients who have tumors that return is small. Treatment for patients with recurrentgerm cell tumor will usually be chemotherapy.
Changes to This Summary (08/23/2007)
The PDQcancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Changes were made to this summary to match those made to the health professional version.
To Learn More
Call
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 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.
Web sites and Organizations
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. There are also many other places where people can get materials and information about cancer treatment and services. Local hospitals may have information on local and regional agencies that offer information about finances, getting to and from treatment, receiving care at home, and dealing with problems associated with cancer treatment.
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), TTY at 1-800-332-8615.
LiveHelp
The NCI's LiveHelp service, a program available on several of the Institute's Web sites, provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 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
For more information from the NCI, please write to this address:
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), TTY at 1-800-332-8615.
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), TTY at 1-800-332-8615.
The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.
Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.