Overview 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 fetus develops, certain cells form sperm in the testicles or eggs in the ovaries. Sometimes these cells travel to other parts of the body and grow into germ cell tumors. This summary is about germ cell tumors that form in parts of the body that are extracranial (outside the brain). Extracranial germ cell tumors are most common in teenagers 15 to 19 years old.
Tumor Types Extracranial germ cell tumors are grouped into mature teratomas, immature teratomas, or malignant germ cell tumors:
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. They are more likely to become cancer.
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 a hormone called alpha-fetoprotein (AFP). • Germinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (β-hCG). • Choriocarcinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (β-hCG).
Malignant extracranial germ cell tumors are grouped into gonadal and extragonadal.
Gonadal Germ Cell Tumors Gonadal germ cell tumors form in the testicles or ovaries.
Testicular Germ Cell Tumors
Testicular germ cell tumors usually occur before the age of 4 years or in teenagers and young adults.
Testicular germ cell tumors in teenagers and young adults are different from those that form in early childhood. They are more like testicular cancer in adults. Testicular germ cell tumors are divided into two main types, nonseminoma and seminoma. (See the PDQ summary on Testicular Cancer Treatment for more information.)
• Nonseminoma: These tumors are usually large and cause symptoms. They tend to grow and spread more quickly than seminomas. • Seminoma: These tumors make a hormone called beta-human chorionic gonadotropin (β-hCG). They are more sensitive to radiation therapy than nonseminomas.
Boys older than 14 years with testicular germ cell tumors are treated in pediatric cancer centers, but the treatment is similar to that used in adults.
Ovarian Germ Cell Tumors
Ovarian germ cell tumors form in egg-making cells in an ovary. These tumors are more common in teenage girls and young women. Most ovarian germ cell tumors are benign teratomas. (See the PDQ summary on Ovarian Germ Cell Tumors Treatment for more information.)
Extragonadal Extracranial Germ Cell Tumors Extragonadal germ cell tumors form in areas other than the testicles or ovaries.
Most germ cell tumors that are not in the testicles, ovaries, or brain form along the midline of the body. This includes the following:
• 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.
Symptoms Different tumors may cause the following signs and symptoms. Other conditions may cause these same symptoms. Consult a doctor 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:
o Pain or a lump in the abdomen. o Fever. o Constipation. o No menstruation. o Unusual vaginal bleeding.
Treatment Different types of treatments are available for children with extracranial germ cell tumors. Some treatments are standard (the currently used treatment, often referred to as “standard of care”), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to 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.
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. 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 both testicles through an incision (cut) in the groin. • Unilateral salpingo-oophorectomy: Surgery to remove one ovary and one fallopian tube.
Watchful waiting Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. For childhood extracranial germ cell tumors, this includes physical exams, imaging tests, and tumor marker tests.
Chemotherapy 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 spinal column, 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.
