Extragonadal Germ Cell Tumours

Key Points for This Section

  • Extragonadal germ cell tumours form from developing sperm or egg cells that travel from the gonads to other parts of the body.
  • Age and gender can affect the risk of developing extragonadal germ cell tumours.
  • Possible signs of extragonadal germ cell tumours include chest pain and breathing problems.
  • Imaging and blood tests are used to detect (find) and diagnose extragonadal germ cell tumours.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

Extragonadal germ cell tumours form from developing sperm or egg cells that travel from the gonads to other parts of the body.

“Extragonadal” means outside of the gonads (sex organs). When cells that are meant to form sperm in the testicles or eggs in the ovaries travel to other parts of the body, they may grow into extragonadal germ cell tumours. These tumours may begin to grow anywhere in the body but usually begin in organs such as the pineal gland in the brain, in the mediastinum, or in the abdomen.

Extragonadal germ cell tumours can be benign (noncancer) or malignant (cancer). Benign extragonadal germ cell tumours are called benign teratomas. These are more common than malignant extragonadal germ cell tumours and often are very large.

Malignant extragonadal germ cell tumours are divided into two types, nonseminoma and seminoma. Nonseminomas tend to grow and spread more quickly than seminomas. They usually are large and cause symptoms. If untreated, malignant extragonadal germ cell tumours may spread to the lungs, lymph nodes, bones, liver, or other parts of the body.

For information about germ cell tumours in the ovaries and testicles, see the following summaries:

  • Ovarian Germ Cell Tumours Treatment
  • Testicular Cancer Treatment

Age and gender can affect the risk of developing extragonadal germ cell tumours.

Risk factors for malignant extragonadal germ cell tumours include the following:

  • Being male.
  • Being age 20 or older.
  • Having Klinefelter's syndrome.

Possible signs of extragonadal germ cell tumours include chest pain and breathing problems.

Malignant extragonadal germ cell tumours may cause symptoms as they grow into nearby areas. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Chest pain.
  • Breathing problems.
  • Cough.
  • Fever.
  • Headache.
  • Change in bowel habits.
  • Feeling very tired.
  • Trouble walking.
  • Trouble in seeing or moving the eyes.

Imaging and blood tests are used to detect (find) and diagnose extragonadal germ cell tumours.

The following tests and procedures may be used:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. The testicles may be checked for lumps, swelling, or pain. A history of the patient's health habits and past illnesses and treatments will also be taken.
  • Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
  • Serum tumour marker test: A procedure in which a sample of blood is examined to measure the amounts of certain substances released into the blood by organs, tissues, or tumour cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumour markers. The following three tumour markers are used to detect extragonadal germ cell tumour:
    • o Alpha-fetoprotein (AFP).
    o Beta-human chorionic gonadotropin (β-hCG).
    o Lactate dehydrogenase (LDH).
    Blood levels of the tumour markers help determine if the tumour is a seminoma or nonseminoma.

    • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerised tomography, or computerised axial tomography.
    • Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the testicles, and make echoes. The echoes form a picture of body tissues called a sonogram.
    • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The type of biopsy used depends on where the extragonadal germ cell tumour is found.
      • o Excisional biopsy: The removal of an entire lump or suspicious tissue.
      o Incisional biopsy: The removal of part of a lump or suspicious tissue.
      o Core biopsy: The removal of part of a lump or suspicious tissue using a wide needle.
      o Needle biopsy or fine-needle aspiration biopsy: The removal of part of a lump, suspicious tissue, or fluid using a thin needle.

      Certain factors affect prognosis (chance of recovery) and treatment options.

      The prognosis (chance of recovery) and treatment options depend on the following:

      • Whether the tumour is nonseminoma or seminoma.
      • The size of the tumour and where it is in the body.
      • The blood levels of AFP, β-hCG, and LDH.
      • Whether the tumour has spread to other parts of the body.
      • The way the tumour responds to initial treatment.
      • Whether the tumour has just been diagnosed or has recurred (come back).

      Stages of Extragonadal Germ Cell Tumours

      Key Points for This Section

      • After an extragonadal germ cell tumour has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.
      • The following prognostic groups are used for extragonadal germ cell tumours:
        • o Good prognosis
        o Intermediate prognosis
        o Poor prognosis

        After an extragonadal germ cell tumour has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

        The extent or spread of cancer is usually described as stages. For extragonadal germ cell tumours, prognostic groups are used instead of stages. The tumours are grouped according to how well the cancer is expected to respond to treatment. It is important to know the prognostic group in order to plan treatment.

        The following prognostic groups are used for extragonadal germ cell tumours:

        Good prognosis

        A nonseminoma extragonadal germ cell tumour is in the good prognosis group if:

        • the tumour is in the back of the abdomen; and
        • the tumour has not spread to organs other than the lungs; and
        • the levels of tumour markers AFP and β-hCG are normal and LDH is slightly above normal.

        A seminoma extragonadal germ cell tumour is in the good prognosis group if:

        • the tumour has not spread to organs other than the lungs; and
        • the level of AFP is normal; β-hCG and LDH may be at any level.

        Intermediate prognosis

        A nonseminoma extragonadal germ cell tumour is in the intermediate prognosis group if:

        • the tumour is in the back of the abdomen; and
        • the tumour has not spread to organs other than the lungs; and
        • the level of any one of the tumour markers (AFP, β-hCG, or LDH) is more than slightly above normal.

        A seminoma extragonadal germ cell tumour is in the intermediate prognosis group if:

        • the tumour has spread to organs other than the lungs; and
        • the level of AFP is normal; β-hCG and LDH may be at any level.

        Poor prognosis

        A nonseminoma extragonadal germ cell tumour is in the poor prognosis group if:

        • the tumour is in the chest; or
        • the tumour has spread to organs other than the lungs; or
        • the level of any one of the tumour markers (AFP, β-hCG, or LDH) is high.

        Seminoma extragonadal germ cell tumour does not have a poor prognosis group.

        Treatment Option Overview

        Key Points for This Section

        • There are different types of treatment for patients with extragonadal germ cell tumours.
        • Three types of standard treatment are used:
          • o Radiation therapy
          o Chemotherapy
          o Surgery
        • New types of treatment are being tested in clinical trials. These include the following:
          • o High-dose chemotherapy with stem cell transplant
        • Tumour markers continue to be checked after initial treatment.

        There are different types of treatment for patients with extragonadal germ cell tumours.

        Different types of treatments are available for patients with extragonadal germ cell tumours. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help 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.

        Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

        Three types of standard treatment are used:

        Radiation therapy

        Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

        Chemotherapy

        Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells 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 in the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

        Surgery

        Patients who have benign tumours or tumour remaining after chemotherapy or radiation therapy may need to have surgery.

        New types of treatment are being tested in clinical trials. These include the following:

        High-dose chemotherapy with stem cell transplant

        High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

        Tumour markers continue to be checked after initial treatment.

        After initial treatment, blood levels of AFP and other tumour markers are checked to find out how well the treatment is working.

        Treatment Options for Extragonadal Germ Cell Tumours

        Benign Teratoma

        Treatment of benign teratomas is surgery.
        Seminoma

        Treatment of seminoma extragonadal germ cell tumours may include the following:

        • Radiation therapy for small tumours in one area, followed by watchful waiting if there is tumour remaining after treatment.
        • Chemotherapy for larger tumours or tumours that have spread. If a tumour smaller than 3 centimeters remains after chemotherapy, watchful waiting follows. If a larger tumour remains after treatment, surgery or watchful waiting follow.

        Nonseminoma

        Treatment of nonseminoma extragonadal germ cell tumours may include the following:

        • Combination chemotherapy followed by surgery to remove any remaining tumour.
        • A clinical trial of a new treatment.

        Information about ongoing clinical trials is available from the NCI Web site.
        Recurrent or Refractory Extragonadal Germ Cell Tumours

        Treatment of extragonadal germ cell tumours that are recurrent (come back after being treated) or refractory (do not get better during treatment) may include the following:

        • Chemotherapy.
        • A clinical trial of high-dose chemotherapy with stem cell transplant.
        • A clinical trial of a new treatment.

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