About This Cancer

Germ cell tumors can be benign (noncancerous) or malignant (cancerous). Benign tumors do not spread, but can cause problems by pressing on nearby tissue and organs. Cancerous tumors, like other cancers, can spread to other parts of the body. They are one of the more rare forms of childhood cancers, representing just three percent of pediatric malignancies, and can be cured most of the time. 

Germ cells are cells that develop in the embryo and change and grow to become the reproductive system in males and females. Sometimes, instead of developing properly, these cells can produce tumors. These tumors arise most commonly in the testes in boys and the ovaries in girls. These tumors are often referred to as gonadal tumors.

Sometimes, however, germ cell tumors occur at the bottom of the spine (sacrococcygeal tumor); in the middle of the brain (pineal gland tumor); the chest cavity (mediastinum tumor); or abdomen (presacral tumor). These tumors are referred to as extragonadal tumors. Other names given to germ cell tumors are yolk sac tumor, dysgerminomas, and teratomas.

Risk Factors

The exact cause of germ cell tumors is unknown.  However, certain inherited disorders have been associated with an increased risk of developing this type of tumor. Some genetic syndromes caused by extra or missing sex chromosomes can cause incomplete or abnormal development of the reproductive system, and an increased risk of germ cell tumors.

Infants born with certain malformations of the central nervous system, genitourinary tract, or lower spine may be at increased risk.  In addition, males with cryptochidism, a condition in which the testes do not descend into the scrotal sac, have an increased risk of developing testicular germ cell tumors.

Cancer Symptoms

Symptoms may be different based on the size and location of the tumor.  They may include swelling or a mass that can be seen or felt. Boys may have an abnormal shape or irregular size of the testicles, elevated blood levels of alpha-fetoprotein (AFP), elevated blood levels of beta-human chorionic gonadotropin (B-HCG).  Constipation, incontinence, and leg weakness  are other symptoms of germ cell tumors.

Gonadal Germ Cell Tumors:

Ovarian tumors:  These tumors are usually not detected until age ten or older. They can grow to a large size before they produce symptoms, though swelling of the abdomen can occur in some cases.

Testicular tumors:  Testicular tumors are often detected at an early stage because they are visible in the scrotum and often cause pain.

Extragonadal Germ Cell Tumors:

Mediastinum tumor (chest cavity):  Tumors in this area may cause chest pain, breathing difficulties, cough, and fever.

Presacral tumors  (abdomen):  A mass may be felt or seen in the lower abdomen or buttocks of an infant or young child. These tumors can cause difficulty in passing urine or having bowel movements, and, in older children, trouble walking. In children under six months, these tumors are usual benign, but, in those older than six months, they are typically cancerous.

Pineal Gland tumors (middle of brain):  The pineal gland is a pea-sized gland in the middle of the brain. Tumors here cause problems by pressing on other parts of the brain or by interfering with the normal flow of cerebrospinal fluid, the fluid that circulates around the brain and spinal cord. Symptoms may include headache, nausea, vomiting, memory loss, fatigue, difficulty walking, inability to look upward, uncontrolled eye movements, or double vision. Sometimes these tumors produce hormones causing the onset of puberty at a very young age.

Sacrococcygeal tumors (base of spine/lower back):  This type of tumor most commonly occurs in newborns. The tumor is usually visible from outside of the body, so diagnosis is usually made very early.

Diagnostic Tests

Diagnostic tests usually include a biopsy, in which a piece of the tumor is removed and examined under a microscope. A CT (computerized tomography) scan and/or an MRI (magnetic resonance imaging) scan is commonly performed to determine the size, exact position, and location of the tumor within the body. These tumors often produce proteins, called alpha-fetoprotein (AFP) and beta-human chorionic gonadotrophin (B-HCG), which can be measured in the blood. As a result, the doctor will order blood tests as well.

Treatment

For benign (noncancerous) germ cell tumors, most patients are cured by the surgical removal of the tumor.  In gonadal germ cell tumors, it may be necessary for the surgeon to remove the testis or ovary where the tumor is located.  

If the tumor is malignant (cancerous), often it can be surgically removed as well.  However, since cancers can spread to other parts of the body, patients with cancerous germ cell tumors are almost always given chemotherapy (treatment with anticancer drugs) following surgery to kill any remaining cancer cells.  Germ cell tumors usually respond very well to chemotherapy, and treatment is usually given every three weeks for four to five months.  Overall cure rates are 90 percent or higher for low-stage patients.