Extracranial Germ Cell Tumors (Childhood)
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 on how the tumor is treated. The major types of germ cell tumors by location and age are:
Testicular germ cell tumors of early childhood
This type of germ cell tumor forms within the testes 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 Testicular Cancer Treatment 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 Ovarian Germ Cell Tumor Treatment 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.
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 cancer cells 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. The following stages are used for all extracranial germ cell tumors with the exception of ovarian germ cell tumors:
Cancer has not spread from the tumor to surrounding tissues or lymph nodes and can be surgically removed with no cancer cells remaining.
Cancer has spread to surrounding tissues or lymph nodes, and surgery cannot remove all cancer cells from the surrounding tissues.
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.
Cancer has spread to other organs in the body.
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.
The following stages are used for ovarian germ cell tumor:
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.
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 abdominal fluid.
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.
Cancer is found in one or both ovaries and has spread outside the abdomen or has spread to the inside of the liver.
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 pediatric oncologist, 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:
-surgery (cutting the tumor out of the body)
-chemotherapy (using drugs to kill tumor cells)
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.
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 malignant testicular germ 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 Testicular Cancer Treatment 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:
-unilateral salpingo-oophorectomy: taking out the ovary with the cancer and the fallopian tube on the same side
-tumor debulking: taking out as much of the cancer as possible
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 Ovarian Germ Cell Tumor Treatment 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 recurrent germ cell tumor will usually be chemotherapy.
The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals.
The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation.
The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. Any use of the information will be at the risk of the user.
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