Mariah
Osteosarcoma Survivor
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Josh
Brain Tumor Survivor
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Sydney
Leukemia Survivor
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Alijah
Leukemia Survivor
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Rosie
Wilms Tumor Survivor
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Ryan
Leukemia Survivor
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Sydney
Retinoblastoma Survivor
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Germ Cell Tumors

Researchers look for a genetic source of childhood tumors

Germ cell tumors (GCTs) are masses of tissue formed by the type of immature cells which, in a healthy body, would develop into mature eggs (in a female) or sperm (in a male). Germ cell tumors may be malignant, which means cancerous, or they may be benign, which means noncancerous.

Quick facts

  • Some GTCs are cancerous, but others are not.
  • Approximately three percent of childhood cancers are malignant germ cell tumors.
  • Ninety percent of germ cell tumors are gonadal, which means they begin in the reproductive cells of the testes or ovaries. The rest are termed extragonadal.
  • GCTs can develop in the central nervous system (CNS), including the brain. GCTs that occur outside the brain are called extracranial.

There are many different types of childhood germ cell tumors:

  • Seminomatous or germinomatous tumors. These are the slower-growing GCTs. Malignant seminomatous GCTs respond well to radiation and chemotherapy, giving patients an excellent prognosis.
    • Seminoma: GTCs of the testis.
    • Dysgerminoma: GTCs in the ovary.
    • Germinoma: GTCs of the same histology of seminoma and dysgerminoma occurring somewhere in the central nervous system (CNS), which includes the brain.
  • Non-germinomas (or nonseminomas): These type of GCTs grow faster.
    • Embryonal carcinomas. These malignant cells are often found in the testicles but can spread elsewhere.
    • Yolk sac tumors (also known as endodermal sinus tumors). These are the most common malignant ovarian and testicular GCTs in children.
    • Choriocarcinomas. These are very rare and often malignant.
    • Teratomas. A teratoma may contain several different types of tissue, such as hair, muscle, and bone. Teratomas of the tailbone are the most common GCT found in children.
  • Mixed germ cell tumors. A mixed germ cell tumor has both types of germ cells in it.

Symptoms

  • A solid mass or lump.
  • Abdominal pain.
  • Chest pain.
  • Excessive hair growth.
  • Early entry into puberty.
  • Frequent urination.

Diagnosis

If the doctor suspects a germ cell tumor is causing the child’s symptoms, the following tests and procedures may be used to learn more:

  • Biopsy: A sample of the patient’s tissue is removed and checked for signs of cancer under a microscope. The sample may be taken during surgery to remove or view the tumor.
  • Blood test: A blood sample taken from the child may be used to run blood chemistry studies or a serum tumor marker test.
  • Immunohistochemistry study: In this laboratory test,  a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
  • X ray: An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body.
  • CT scan (computerized tomography): In this procedure, detailed pictures of the body are taken from different angles by a computer linked to an x-ray machine. Dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly in the image.
  • MRI (magnetic resonance imaging): For this imaging procedure, magnet, radio waves, and a computer make a series of detailed pictures of areas inside the body.
  • Ultrasound: High-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes in this procedure. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
  • Bone scan: The patient is injected with a small amount of radioactive material, referred to as a “tracer,” for this imaging procedure. A scanner notes the places in the body where the tracer accumulates, which may indicate the location of a cancer tumor.

Staging

If doctors diagnose a GCT, the next step is to determine how far the cancer has spread in the body. Additional tests may be needed, such as:

  • Thoracentesis: The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.
  • Paracentesis: The removal of fluid from the space between the lining of the abdomen and the organs in the abdomen, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.

Staging of GCTs of the central nervous system (CNS), including the brain: In childhood brain and spinal cord tumors, there is no standard staging system. The plan for cancer treatment depends on several factors:

  • The type of tumor and where the tumor formed in the brain.
  • Whether the tumor is newly diagnosed or has come back after treatment (recurrent).
  • The tumor’s “grade,” which is based on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread.
  • The tumor risk group, based on the amount of tumor remaining after surgery, the spread of cancer cells within the brain and spinal cord or to other parts of the body, where the tumor has formed, and the age of the child.

Staging of childhood nonseminoma testicular GCTs:

  • Stage I: The cancer is found only in the testicle and is completely removed by surgery. Tumor marker levels return to normal after surgery.
  • Stage II: The cancer is removed by surgery and some cancer cells remain in the scrotum, or cancer that can only be seen with a microscope has spread to the scrotum or spermatic cord. Tumor marker levels do not return to normal after surgery and may increase.
  • Stage III: The cancer has spread to one or more lymph nodes in the abdomen and is not completely removed by surgery. The cancer that remains after surgery can be seen without a microscope.
  • Stage IV: The cancer has spread to distant parts of the body, such as the liver.

Staging of childhood ovarian GCTs:

  • Stage I: The cancer is in the ovary and can be completely removed by surgery.
  • Stage II: One of the following is true of the cancer:
    • It is not completely removed by surgery. The remaining cancer can be seen with a microscope only.
    • It has spread to the lymph nodes and can be seen with a microscope only.
    • It has spread to the capsule (outer covering) of the ovary.
  • Stage III: One of the following is true of the cancer:
    • It is not completely removed by surgery. The remaining cancer can be seen without a microscope.
    • It has spread to lymph nodes and the lymph nodes are 2 centimeters or larger.
    • It is found in fluid in the abdomen.
  • Stage IV: The cancer has spread to the lung, liver, brain, or bone.

Staging of extragonadal extracranial GCTs:

  • Stage I: The cancer is in one place and can be completely removed by surgery. For tumors at the base of the tailbone, the cancer and tailbone are completed removed by surgery. Tumor marker levels return to normal after surgery.
  • Stage II: The cancer has spread to nearby tissues and/or lymph nodes and is not completely removed by surgery. The cancer remaining after surgery can be seen with a microscope only. Tumor marker levels do not return to normal after surgery and may increase.
  • Stage III: One of the following is true of the cancer:
    • It is not completely removed by surgery. The cancer remaining after surgery can be seen without a microscope.
    • It has spread to lymph nodes and is larger than 2 centimeters in diameter.
    • It is found in fluid in the abdomen.
  • Stage IV: The cancer has spread to distant parts of the body, including the liver.

Treatment

Treatment for childhood GCT may include a standard treatment or a treatment clinical trial. Clinical trials are part of the cancer research process. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Types of standard treatment used include:

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
In chemotherapy, drugs are taken orally or injected to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Combination chemotherapy is treatment using more than one anticancer drug.

Radiation therapy

High-energy x-rays or other types of radiation kill cancer cells or keep them from growing in this therapy, which can involve a machine sending radiation towards the body (external) or a radioactive substance sealed in needles, seeds, wires, or catheters being placed near the cancer (internal).

Treatment by type of GCT

For GCTs of the central nervous system (CNS), including brain tumors, treatment may include:

  • Radiation therapy, usually to the whole brain and spine.
  • Chemotherapy.

For childhood extracranial GCTs, treatment may include:

  • Mature and immature teratomas in the sacrum or coccyx: Treatment is usually surgery followed by watchful waiting. Most teratomas can be removed completely. If the tumor is in the coccyx, the entire coccyx is removed. Chemotherapy may be given if the tumor comes back.
  • Mature and immature teratomas that are not in the sacrum or coccyx: Treatment is usually surgery followed by watchful waiting. A second surgery may be done to remove any remaining cancer.
  • Regular follow-up exams with imaging tests and the alpha-fetoprotein (AFP) tumor marker test will be done for at least 3 years.

For malignant testicular GCTs, treatment may include:

  • For boys younger than 15 years:
    • Surgery (radical inguinal orchiectomy) followed by watchful waiting for stage I tumors. Chemotherapy may be given if the tumor comes back.
    • Surgery (radical inguinal orchiectomy) followed by combination chemotherapy for stage II-IV tumors.
    • A clinical trial of lower doses and fewer cycles of chemotherapy after surgery.

     

  • For boys 15 years and older:
    • Malignant testicular germ cell tumors in boys 15 years and older are treated differently than they are in young boys. Surgery may include removal of lymph nodes in the abdomen. Treatment depends on whether the tumor is a seminoma or a nonseminoma.

For malignant ovarian GCTs, treatment may include:

  • Surgery (unilateral salpingo-oophorectomy) followed by watchful waiting for stage I dysgerminoma or immature teratoma tumors. Chemotherapy may be given if the tumor comes back.
  • Surgery (unilateral salpingo-oophorectomy) followed by combination chemotherapy for stages II-IV tumors.
  • Chemotherapy to make the tumor smaller, followed by surgery (unilateral salpingo-oophorectomy).
  • Surgery (tumor debulking) followed by chemotherapy.
  • A clinical trial of lower doses and fewer cycles of chemotherapy after surgery.
  • A clinical trial of surgery (unilateral salpingo-oophorectomy) followed by watchful waiting for stage I tumors that are not a dysgerminoma or immature teratoma.

For malignant extragonadal GCTs, treatment may include:

  • Surgery to remove tumors in the mediastinum (the area between the lungs).
  • Surgery followed by combination chemotherapy.
  • Combination chemotherapy followed by surgery.
  • Chemotherapy only.
  • A clinical trial of lower doses and fewer cycles of chemotherapy.
  • A clinical trial of a new chemotherapy regimen.

For recurrent childhood malignant extracranial GCTs, there is no standard treatment. Treatment is usually within in a clinical trial and may include the following:

  • Surgery followed by combination chemotherapy, for malignant testicular germ cell tumors that recur after being treated with surgery and watchful waiting.
  • Surgery for tumors that come back in the sacrum or coccyx. Chemotherapy may be given before surgery, to shrink the tumor. If any of the tumor remains after surgery, radiation therapy may be added.
  • Surgery followed by chemotherapy, for malignant ovarian germ cell tumors that recur after being treated with surgery and watchful waiting.

Leading the way with crucial research

As world-renowned leaders in cancer care, the specialists of the University of Minnesota’s Masonic Cancer Center are dedicated to curing cancer in all of its forms. Children’s Cancer Research Fund’s relationship with the University of Minnesota supports the work of multidisciplinary teams focusing on specific tumor categories and developing the next generation of cancer therapies. Through clinical trials, patients have access to the most current knowledge, techniques and technology.

Children's Cancer Research Fund sponsorship for germ cell tumor investigations has led to a major grant from the National Institutes of Health. University of Minnesota pediatric cancer epidemiologist Dr. Jen Poynter received a five-year, $3.5 million grant to conduct a comprehensive evaluation of genetic changes that occur in pediatric germ cell tumors. The funding will allow Dr. Poynter and colleagues to begin the largest epidemiologic study ever conducted on the genetic susceptibility of developing pediatric germ cell tumors.

Understanding how GCTs develop may give important insights into the fetal origins of cancer development, leading to improved treatments and prevention of both pediatric and adult tumors. To learn more, visit our Disease Research page.

This information is displayed with permission of the University of Minnesota Cancer Center. For more information about this disease or to review active clinical trials, please visit the website of the Cancer Center.

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