Childhood Extracranial Germ Cell Tumour

What is childhood extracranial germ cell tumour?

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 tumour. This summary covers germ cell tumours that occur extracranially (everywhere but in the brain).

Extracranial germ cell tumours can be benign (not cancerous) or malignant (cancerous). Most germ cell tumours are benign and are very rare in children younger than age 15. Germ cell tumours of early childhood have biological characteristics which are different than those that occur in adolescents and young adults. The location of the tumour and the age of the child make a difference on how the tumour is treated. The major types of germ cell tumours by location and age are:
Testicular germ cell tumours of early childhood

This type of germ cell tumour forms within the testes of young boys. The treatment for this type of germ cell tumour is covered later in this summary.
Testicular germ cell tumours of adolescence and young adulthood

This type of germ cell tumour forms within the testes of older boys. Testicular germ cell tumours 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 summary on Testicular Cancer Treatment for more information.)
Extragonadal, extracranial germ cell tumours of early childhood

This includes any type of germ cell tumour that is not located in the reproductive organs (testicles or ovaries) or in the brain. These germ cell tumours 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 tumour is covered later in this summary.
Extragonadal, extracranial germ cell tumours of adolescence and young adulthood

This type of germ cell tumour is usually located within the chest. The treatment for this type of germ cell tumour is covered later in this summary.
Ovarian germ cell tumours

Ovarian germ cell tumour, 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 tumour is covered later in this summary (refer to the summary on Ovarian Germ Cell Tumour Treatment for more information).

Germ cell tumours form in developing cells and usually contain tissues that are foreign to the location of the tumour. Germ cell tumours can further be classified as teratomas or malignant germ cell tumours. Teratomas can be either mature (well differentiated tissue that forms a tumour 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 tumours.

Stage Explanation

Once a germ cell tumour is found (diagnosed), tests will be done to find out if the germ cell tumour is benign or malignant. If the tumour 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 tumour, 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 Tumours

The following stages are used for all extracranial germ cell tumours:
Stage I

Cancer has not spread from the tumour 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 tumour 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 tumour or in another place.
Ovarian Germ Cell Tumour

The following stages are used for ovarian germ cell tumour:
Stage I

Cancer is found in either one or both of the ovaries; it has not spread to the surrounding tissue. Tumour 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). Tumour cells may be present in abdominal fluid.
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 tumours. Your child’s treatment will often be coordinated by a paediatric oncologist, a doctor who specializes in cancer in children. The paediatric oncologist may refer your child to other specialists, such as a paediatric surgeon, a psychologist, a radiation oncologist, and other doctors who specialize in the type of treatment your child requires.

Treatment for extracranial germ cell tumour depends upon the location of the tumour, the stage of the tumour, and the type of tumour. The types of treatment used for extracranial germ cell tumour are:

  • Surgery (cutting the tumour out of the body)
  • Chemotherapy (using drugs to kill tumour cells)

More than one method of treatment may be used, depending on the type of extracranial germ cell tumour and how much cancer the patient has in his or her body. Complete or near complete surgical removal of the tumour is often possible. If the tumour 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 tumour is a mature or immature teratoma. If the tumour is a mature teratoma, the treatment will be surgery to remove the tumour and possibly some of the surrounding tissues or structures. If the tumour is an immature teratoma, treatment will be surgery with or without chemotherapy.

Childhood Malignant Testicular Germ Cell Tumour

Treatment will depend on the age of the child. The majority of childhood malignant testicular germ cell tumours occur in boys younger than 4 years of age. Surgery is the most common form of treatment for testicular germ cell tumour. A doctor may take out the tumour 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 tumour is the same as the treatment for adults. (Refer to the summary on Testicular Cancer Treatment for more information.)

Childhood Malignant Ovarian Germ Cell Tumour

Treatment will depend on the age of the child and the specific tumour type. Surgery is the most common form of treatment for ovarian germ cell tumour. 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
  • Tumour debulking: taking out as much of the cancer as possible

Treatment for young girls with early stage ovarian germ cell tumour will be unilateral salpingo-oophorectomy. Treatment for young girls with advanced stage ovarian germ cell tumour will be unilateral salpingo-oophorectomy with or without chemotherapy.

The treatment for adolescents and young adults with ovarian germ cell tumour is similar to the treatment for adults. (Refer to the summary on Ovarian Germ Cell Tumour Treatment for more information.)

Childhood Extragonadal Malignant Germ Cell Tumour

Treatment for childhood extragonadal malignant germ cell tumour depends on the size and location of the tumour. Treatment for smaller tumours will be surgery to remove the tumour followed by chemotherapy. Treatment for larger tumours will be a biopsy (a surgical procedure to remove a small portion of the tumour) followed by chemotherapy to reduce the size of the tumour, possibly followed by surgery to remove any remaining tumour.

Recurrent Childhood Malignant Germ Cell Tumour

Due to the small number of childhood extracranial germ cell tumours and the effectiveness of treatment, the number of patients who have tumours that return is small. Treatment for patients with recurrent germ cell tumour will usually be chemotherapy.


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