Malignant mesothelioma is a cancer of the thin tissue (mesothelium) that lines the lung, chest wall, and abdomen. The major risk factor for mesothelioma is asbestos exposure. Explore the links on this page to learn more about malignant mesothelioma treatment and clinical trials.
Peritoneum: A thin layer of tissue that lines the abdomen and covers most of the organs in the abdomen.
Pericardium: A thin layer of tissue that surrounds the heart.
The tumors often spread over the surface of organs without spreading into the organ. They may spread to nearby lymph nodes or in other parts of the body. Malignant mesothelioma may also form in the testicles, but this is rare.
EnlargeMalignant mesothelioma forms in the tissue that lines the chest or abdomen, including the pleura (the tissue that lines the chest cavity and covers the lungs), the pericardium (the tissue that surrounds the heart), and the peritoneum (the tissue that lines the abdomen and covers most of the organs in the abdomen). Malignant mesothelioma may also form in the testicles, but this is rare.
Treatment with radiation therapy increases the risk of childhood mesothelioma.
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk.
In adults, mesothelioma is strongly linked to being exposed to asbestos, which has been used in the building and textile industries. In children, there is little information about the risk of developing mesothelioma after being exposed to asbestos.
Signs and symptoms of mesothelioma include trouble breathing and pain in the chest or abdomen.
In children, these and other signs and symptoms may be caused by mesothelioma or by other conditions.
Check with your child’s doctor if your child has any of the following:
Trouble breathing.
Cough for no known reason.
Pain under the rib cage or pain in the chest and abdomen.
Weight loss for no known reason.
Feeling very tired.
Tests that examine the chest, abdomen, and heart are used to diagnose mesothelioma.
The following tests and procedures may be used:
Physical exam and health 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. 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.
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, computerized tomography, or computerized axial tomography.
PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. EnlargePositron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child’s vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Pulmonary function test (PFT): A test to see how well the lungs are working. It measures how much air the lungs can hold and how quickly air moves into and out of the lungs. It also measures how much oxygen is used and how much carbon dioxide is given off during breathing. This is also called a lung function test.
Thoracoscopy: A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung.
Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for abnormal areas. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of cancer.
Cytologic exam: An exam of cells under a microscope (by a pathologist) to check for anything abnormal. For mesothelioma, fluid is taken from around the lungs or from the abdomen. A pathologist checks the cells in the fluid.
Certain factors affect prognosis (chance of recovery).
The process used to find out if cancer has spread from where it first began is called staging. In childhood mesothelioma, cancer may spread to nearby or distant lymph nodes. There is no standard staging system for childhood mesothelioma. The results of tests and procedures done to diagnose mesothelioma are used to help make decisions about treatment.
Sometimes childhood mesothelioma recurs (comes back) after it has been treated.
Treatment Option Overview
Key Points
There are different types of treatment for children with mesothelioma.
Children with mesothelioma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Three types of treatment are used:
Surgery
Chemotherapy
Radiation therapy
New types of treatment are being tested in clinical trials.
Targeted therapy
Immunotherapy
Treatment for childhood mesothelioma may cause side effects.
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
There are different types of treatment for children with mesothelioma.
Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. 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.
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.
Children with mesothelioma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:
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).
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.
Targeted therapy is being studied for the treatment of childhood mesothelioma that has recurred (come back).
Immunotherapy
Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy.
Treatment for childhood mesothelioma may cause side effects.
Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
Physical problems.
Changes in mood, feelings, thinking, learning, or memory.
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
A clinical trial that checks a sample of the patient’s tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood mesothelioma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary (“Updated”) is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Mesothelioma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/mesothelioma/patient/child-mesothelioma-treatment-pdq. Accessed <MM/DD/YYYY>.
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Childhood cervical and vaginal cancers are very rare types of cancer. Cervical cancer forms in the cells of the cervix, and vaginal cancer forms in the cells of the vagina. The cervix is the lower, narrow end of the uterus (the hollow, pear-shaped organ where a fetus grows). The cervix leads from the uterus to the vagina (birth canal). The vagina is the canal leading from the cervix to the outside of the body. At birth, a baby passes out of the body through the vagina. Learn more about how cancer develops at What Is Cancer?
EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium.
Childhood cervical cancer and vaginal cancer symptoms
The most common symptom of cervical cancer and vaginal cancer in children is bleeding from the vagina. Other conditions may also cause vaginal bleeding. If your child has vaginal bleeding, it is important that you tell their doctor. The doctor will ask you when it started and how often it occurs as a first step in making a diagnosis.
Tests to diagnose childhood cervical cancer and vaginal cancer
If your child has symptoms that suggest vaginal or cervical cancer, their doctor will need to find out if they are due to cancer or another problem. They may ask about your child’s personal and family medical history and do a physical exam.
Depending on your child’s symptoms and medical history and the results of their physical exam, the doctor may recommend more tests to find out if your child has vaginal or cervical cancer, and if so, its extent (stage). The results of tests and procedures done to diagnose vaginal and cervical cancers are used to help make decisions about treatment.
The following tests and procedures may be used to diagnose and stage childhood cervical or vaginal cancers:
Pap test
The Pap test (also called a Pap smear or cervical cytology) collects cervical cells from the surface of the cervix and vagina using a soft, narrow brush or tiny spatula. The cells are viewed under a microscope to find out if they are abnormal.
Biopsy
Transvaginal needle biopsy is the removal of tissue using a needle that is guided by ultrasound. A pathologist views the tissue under a microscope to check for signs of cancer.
Serum tumor marker test
Serum tumor markers are substances found in the blood that are either made by vaginal or cervical cancer cells or that the body makes in response to vaginal or cervical cancer. For this test, a sample of blood is checked in the lab to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body.
Ultrasound exam
An ultrasound exam uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs, such as the pelvis, and make echoes. The echoes form a picture of body tissues called a sonogram.
Magnetic resonance imaging (MRI)
MRI uses a magnet and radio waves to make a series of detailed pictures of areas inside the body, such as the pelvis. The pictures are made by a computer. This procedure is also called nuclear magnetic resonance imaging.
CT scan (CAT scan)
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the pelvis. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
Getting a second opinion
You may want to get a second opinion to confirm your child’s cervical or vaginal cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s cancer.
To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, see Questions to Ask Your Doctor about Cancer.
Cervical cancer and vaginal cancer stages
Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. It is important to know the stage of the cervical or vaginal cancer to plan the best treatment.
There are several staging systems for cancer that describe the extent of the cancer. The International Federation of Gynecology and Obstetrics (FIGO) staging system is used for cervical and vaginal cancers. You may see your child’s cancer described by this staging system in the pathology report. Based on the FIGO results, a stage is assigned to the cancer, ranging from stage I, stage II, stage III, or stage IV (may also be written as stage 1, stage 2, stage 3, or stage 4). When talking with you, your child’s doctor may describe it as one of these stages.
Recurrent cancer is cancer that has come back after it has been treated. Cervical or vaginal cancer may come back in the cervix or vagina or as metastatic tumors in other parts of the body. Tests will be done to help determine where the cancer has returned in the body, if it has spread, and how far. The type of treatment that your child will have for recurrent cancer will depend on how far it has spread.
Types of treatment for childhood cervical and vaginal cancer
There are different types of treatment for children and adolescents with cervical cancer or vaginal cancer. You and your child’s cancer care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the cancer is newly diagnosed or has come back.
A pediatric oncologist, a doctor who specializes in treating children with cancer, will oversee treatment for childhood cervical and vaginal cancers. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:
Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s cancer care team before treatment begins about what to expect. For help every step of the way, see our downloadable booklet, Children with Cancer: A Guide for Parents.
A cervical cancer diagnosis can raise concerns about whether treatment will affect your child’s fertility. Talk with your child’s cancer care team before treatment begins about what to expect. For more information and support, see Fertility Issues in Girls and Women with Cancer.
Types of treatment your child might have include:
Surgery
Surgery is used to remove as much cancer as possible from the cervix or vagina. If cancer cells remain after surgery or cancer has spread to the lymph nodes, more treatment may be needed.
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Cervical cancer and vaginal cancer are sometimes treated with external beam radiation therapy. This type of radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Radiation therapy may be given alone or with other types of treatment, such as chemotherapy.
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cells or stops them from dividing. Chemotherapy for vaginal cancer or cervical cancer is injected into a vein. When given this way, the drugs enter the bloodstream to reach cancer cells throughout the body.
It is not known if chemotherapy is an effective treatment for childhood cervical cancer or vaginal cancer, although drugs commonly used to treat these cancers in adults, such as carboplatin and paclitaxel, may be used.
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Treatment of newly diagnosed childhood cervical cancer and vaginal cancer
Treatment of newly diagnosed cervical cancer and vaginal cancer in children may include:
Surgery will be done to remove as much of the cancer as possible, followed by radiation therapy, if cancer cells remain after surgery or cancer has spread to the lymph nodes.
Chemotherapy may also be used, but it is not yet known how well this treatment works.
Sometimes childhood cervical cancer and vaginal cancer can recur (come back) after treatment. If your child is diagnosed with a recurrent cervical cancer or vaginal cancer, your child’s doctor will work with you to plan treatment.
Side effects and late effects of treatment
Cancer treatment can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Physical problems, such as problems with fertility, may be a late effect of treatment.
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. Learn more about Late Effects of Treatment for Childhood Cancer.
Follow-up care
Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Coping with your child's cancer
When a child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is also important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, see Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.
A cancer stage describes the extent of cancer in the body, especially whether the cancer has spread from where it first formed to other parts of the body. It is important to know the stage of cervical cancer in order to plan the best treatment.
In stage I, cervical cancer has formed and is found in the cervix only. It is divided into stages IA and IB, based on the size of the tumor and the deepest point of tumor invasion.
Stage IA is subdivided based on the deepest point of tumor invasion.
EnlargeStage IA1 and IA2 cervical cancer. A very small amount of cancer that can only be seen under a microscope is found in the tissues of the cervix. In stage IA1, the cancer is not more than 3 millimeters deep. In stage IA2, the cancer is more than 3 but not more than 5 millimeters deep.
Stage IA1: A very small amount of cancer that can only be seen with a microscope is found in the tissues of the cervix. The deepest point of tumor invasion is 3 millimeters or less.
Stage IA2: A very small amount of cancer that can only be seen with a microscope is found in the tissues of the cervix. The deepest point of tumor invasion is more than 3 millimeters but not more than 5 millimeters. EnlargeMillimeters (mm). A sharp pencil point is about 1 mm, a new crayon point is about 2 mm, and a new pencil eraser is about 5 mm.
Stage IB is subdivided based on the size of the tumor and the deepest point of tumor invasion.
Stage IB1: The tumor is 2 centimeters or smaller and the deepest point of tumor invasion is more than 5 millimeters. EnlargeStage IB1 cervical cancer. The cancer is 2 centimeters or smaller and is more than 5 millimeters deep.
Stage IB2: The tumor is larger than 2 centimeters but not larger than 4 centimeters. EnlargeStage IB2 and IB3 cervical cancer. In stage IB2, the cancer is larger than 2 centimeters but not larger than 4 centimeters. In stage IB3, the cancer is larger than 4 centimeters.
Stage IB3: The tumor is larger than 4 centimeters. EnlargeTumor sizes are often measured in centimeters (cm) or inches. Common food items that can be used to show tumor size in cm include: a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm or 2 inches), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm or 4 inches).
In stage II, cervical cancer has spread to the upper two-thirds of the vagina or to the tissue around the uterus.
Stage II is subdivided based on how far the cancer has spread.
EnlargeStage II cervical cancer. In stages IIA1 and IIA2, cancer has spread from the cervix to the upper two-thirds of the vagina but has not spread to the tissue around the uterus. In stage IIA1, the cancer is 4 centimeters or smaller. In stage IIA2, the cancer is larger than 4 centimeters. In stage IIB, cancer has spread from the cervix to the tissue around the uterus.
Stage IIA: Cancer has spread from the cervix to the upper two-thirds of the vagina but has not spread to the tissue around the uterus. Stage IIA is further divided based on the size of the tumor:
Stage IIA1: The tumor is 4 centimeters or smaller.
Stage IIA2: The tumor is larger than 4 centimeters.
Stage IIB: Cancer has spread from the cervix to the tissue around the uterus.
In stage III, cervical cancer has spread to the lower third of the vagina and/or to the pelvic wall, and/or has caused kidney problems, and/or involves lymph nodes.
Stage III is subdivided based on how far the cancer has spread.
Stage IIIA: Cancer has spread to the lower third of the vagina but has not spread to the pelvic wall. EnlargeStage IIIA cervical cancer. Cancer has spread to the lower third of the vagina but has not spread to the pelvic wall.
Stage IIIB: Cancer has spread to the pelvic wall; and/or the tumor has become large enough to block one or both ureters or has caused one or both kidneys to get bigger or stop working. EnlargeStage IIIB cervical cancer. Cancer has spread to the pelvic wall and/or the tumor has become large enough to block one or both ureters or has caused one or both kidneys to get bigger or stop working.
Stage IIIC: Stage IIIC is divided into stages IIIC1 and IIIC2, based on the spread of cancer to the lymph nodes. EnlargeStage IIIC cervical cancer. In stage IIIC1, cancer has spread to lymph nodes in the pelvis. In stage IIIC2, cancer has spread to lymph nodes in the abdomen near the aorta.
In stage IV, cervical cancer has spread beyond the pelvis, or has spread to the lining of the bladder or rectum, or has spread to other parts of the body.
Stage IV is subdivided into stages IVA and IVB, based on where the cancer has spread.
Stage IVA: Cancer has spread to nearby pelvic organs, such as the bladder or rectum. EnlargeStage IVA cervical cancer. Cancer has spread to nearby pelvic organs, such as the bladder or rectum.
Stage IVB: Cancer has spread to other parts of the body, such as the liver, lungs, bones, or distant lymph nodes. EnlargeStage IVB cervical cancer. Cancer has spread to other parts of the body, such as the lymph nodes, lung, liver, or bone.
Stage IV is also called metastatic cancer. Metastatic cancer happens when cancer cells travel through the lymphatic system or blood and form tumors in other parts of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if cervical cancer spreads to the lung, the cancer cells in the lung are actually cervical cancer cells. The disease is called metastatic cervical cancer, not lung cancer. Learn more in Metastatic Cancer: When Cancer Spreads.
Recurrent cervical cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the cervix or as metastatic tumors in other parts of the body. Tests will be done to help determine where the cancer has returned in your body, if it has spread, and how far. The type of treatment that you have for recurrent cervical cancer will depend on how far it has spread.
When cancer comes back, you have the experience to face it that you didn’t have before.
Credit: iStock
When cancer comes back after treatment, doctors call it a recurrence or recurrent cancer. Finding out that cancer has come back can cause feelings of shock, anger, sadness, and fear. But you have something now that you didn’t have before—experience. You’ve lived through cancer already and you know what to expect. Also, remember that treatments may have improved since you were first diagnosed. New drugs or methods may help with your treatment or in managing side effects. In some cases, improved treatments have helped turn cancer into a chronic disease that people can manage and live with for many years.
Why Cancer Comes Back
Recurrent cancer starts with cancer cells that the first treatment didn’t fully remove or destroy. This doesn’t mean that the treatment you received was wrong. It just means that a small number of cancer cells survived the treatment and were too small to show up in follow-up tests. Over time, these cells grew into tumors or cancer that your doctor can now detect.
Sometimes, a new type of cancer will occur in people who have a history of cancer. When this happens, the new cancer is known as a second primary cancer. Second primary cancer is different from recurrent cancer.
Types of Recurrent Cancer
Doctors describe recurrent cancer by where it develops and how far it has spread. The different types of recurrence are:
Local recurrence means that the cancer is in the same place as the original cancer or very close to it.
Regional recurrence means that the tumor has grown into lymph nodes or tissues near the original cancer.
Distant recurrence means the cancer has spread to organs or tissues far from the original cancer. When cancer spreads to a distant place in the body, it is called metastasis or metastatic cancer. When cancer spreads, it is still the same type of cancer. For example, if you had colon cancer, it may come back in your liver. But, the cancer is still called colon cancer.
Staging Recurrent Cancer
To figure out the type of recurrence you have, you will have many of the same tests you had when your cancer was first diagnosed, such as lab tests and imaging procedures. These tests help determine where the cancer has returned in your body, if it has spread, and how far. Your doctor may refer to this new assessment of your cancer as “restaging.”
After these tests, the doctor may assign a new stage to the cancer. An “r” will be added to the beginning of the new stage to reflect the restaging. The original stage at diagnosis does not change.
See our information on Diagnosis to learn more about the tests that may be used to assess recurrent cancer.
Treatment for Recurrent Cancer
The type of treatment that you have for recurrent cancer will depend on your type of cancer and how far it has spread. To learn about the treatments that may be used to treat your recurrent cancer, find your type of cancer among the PDQ® cancer treatment summaries for adult and childhood cancers.
Childhood ovarian tumors form in the tissues of the ovaries. Most ovarian tumors in children are not cancer. Ovarian cancer in children is very rare but occurs most often in females aged 15 to 19 years.
The ovaries are a pair of organs in the female reproductive system. They are in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond in an adult woman. The ovaries make eggs and female hormones (chemicals that control the way certain cells or organs work).
EnlargeAnatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium.
Types of childhood ovarian tumors
Several types of ovarian tumors are seen in children:
Epithelial tumors may be benign or cancer. These tumors begin in the tissue covering the ovary. Epithelial tumors are the second most common ovarian tumor in girls. Ovarian epithelial cancer is usually found at an early stage in children and is easier to treat in children than in adults.
Stromal tumors may be benign or cancer. These tumors begin in stromal cells, which make up tissues that surround and support the ovaries. Juvenile granulosa cell tumors and Sertoli-Leydig cell tumors are two types of stromal tumors.
Small cell carcinoma of the ovary begins in the ovary and may spread to the abdomen, pelvis, or other parts of the body. This type of ovarian cancer is fast growing and has a poor prognosis.
This page is about ovarian non-germ cell tumors (epithelial tumors, stromal tumors, and small cell carcinoma of the ovary).
Causes and risk factors for childhood ovarian cancer
Ovarian tumors in children are caused by certain changes to the way ovarian cells function, especially how they grow and divide into new cells. Often, the exact cause of these cell changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Not every child with a risk factor will develop an ovarian tumor. And it will develop in some children who don’t have a known risk factor.
The following disorders increase a child’s risk of juvenile granulosa cell tumors:
Ollier disease is a rare disorder that causes abnormal growth of cartilage at the end of long bones.
Maffucci syndrome is a very rare disorder that causes abnormal growth of cartilage at the end of long bones and benign blood vessel tumors in the skin.
The following disorders increase a child’s risk of Sertoli-Leydig cell tumors:
Peutz-Jeghers syndrome is a genetic disorder that causes polyps to form in the intestines and dark spots to form on the mouth and fingers.
DICER1 syndrome is a rare genetic disorder that may cause a goiter, polyps in the colon, and tumors of the ovary, cervix, testicle, kidney, brain, eye, and lining of the lung.
Talk with your child’s doctor if you think your child may be at risk.
Genetic counseling for children with Sertoli-Leydig cell tumor
It may not be clear from the family medical history whether your child’s ovarian tumor is part of an inherited condition related to a change in the DICER1 gene. Genetic counseling can assess the likelihood that your child’s cancer is inherited and whether genetic testing is needed. Genetic counselors and other specially trained health professionals can discuss your child’s diagnosis and your family’s medical history to help you understand:
the options for DICER1 gene testing
the risk of other cancers for your child
the risk of a Sertoli-Leydig cell tumor or other cancers for your child’s siblings
the risks and benefits of learning genetic information
Genetic counselors can also help you cope with your child’s genetic testing results, including how to discuss the results with family members. They can advise you about whether other members of your family should receive genetic testing.
These symptoms may be caused by problems other than a tumor. The only way to know is to see your child’s doctor.
Tests to diagnose childhood ovarian cancer
If your child has symptoms that suggest they have an ovarian tumor, the doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with an ovarian tumor, the results of these tests will help you and your child’s doctor plan treatment.
The tests and procedures used to diagnose ovarian tumors may include:
CT scan
CT scan (CAT scan) uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. 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, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas in the body, such as the pelvis or abdomen. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Ultrasound
Ultrasound uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
Biopsy
Biopsy is the removal of a sample of cells or tissue from the tumor so that a pathologist can view it under a microscope to check for cancer.
Serum tumor marker test
A serum tumor marker test examines a sample of blood to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The tumor markers alpha-fetoprotein, beta-human chorionic gonadotropin (beta-hCG), CEA, CA-125, and others are used to diagnose ovarian cancer.
DICER1 genetic testing
DICER1 genetic testing examines a sample of blood or saliva for changes in the DICER1 gene. This test may be done for children with a Sertoli-Leydig cell tumor.
Molecular testing
A molecular test checks for certain genes, proteins, or other molecules in a sample of tissue, blood, or bone marrow. Molecular tests also check for certain changes in a gene or chromosome that may cause or affect the chance of developing an ovarian tumor. A molecular test may be used to help plan treatment, find out how well treatment is working, or make a prognosis.
The Molecular Characterization Initiative offers free molecular testing to children, adolescents, and young adults with certain types of newly diagnosed cancer. The program is offered through NCI’s Childhood Cancer Data Initiative. To learn more, visit About the Molecular Characterization Initiative.
Getting a second opinion
You may want to get a second opinion to confirm your child’s diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the genetic test report, pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s tumor.
To learn more about choosing a doctor and getting a second opinion, visit Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, visit Questions to Ask Your Doctor About Cancer.
Stages of childhood ovarian cancer
Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. It is important to know the stage of ovarian cancer to plan the best treatment.
There are several staging systems for cancer. The International Federation of Gynecology and Obstetrics (FIGO) staging system is used for ovarian cancer. Based on the FIGO results, a stage is assigned to your child’s ovarian cancer, such as stage I, stage II, stage III, or stage IV (may also be written as stage 1, stage 2, stage 3, or stage 4). When talking with you about your child’s cancer, your child’s doctor may describe it as one of these stages.
Stage I (also called stage 1) ovarian cancer
EnlargeIn stage IA, cancer is found inside a single ovary or fallopian tube. In stage IB, cancer is found inside both ovaries or fallopian tubes. In stage IC, cancer is found inside one or both ovaries or fallopian tubes and one of the following is true: (a) either the tumor or the capsule (outer covering) of the ovary has ruptured (broken open), or (b) cancer is also found on the surface of the ovary or fallopian tube, or (c) cancer cells are found in the pelvic peritoneal fluid.
In stage I, ovarian cancer has formed and is found in one or both ovaries or fallopian tubes. Stage I is divided into stages IA, IB, and IC.
Stage IA: Cancer is found inside a single ovary or fallopian tube.
Stage IB: Cancer is found inside both ovaries or fallopian tubes.
Stage IC: Cancer is found inside one or both ovaries or fallopian tubes. Stage IC is further divided based on whether the cancer is found outside the ovary.
Stage IC1: The tumor ruptured (broke open) during surgery.
Stage IC2: The capsule (outer covering) of the ovary ruptured before surgery, or there is cancer on the surface of the ovary or fallopian tube.
Stage IC3: Cancer cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity).
Stage II (also called stage 2) ovarian cancer
EnlargeIn stage IIA, cancer is found in one or both ovaries or fallopian tubes and has spread to the uterus and/or the fallopian tubes and/or the ovaries. In stage IIB, cancer is found in one or both ovaries or fallopian tubes and has spread to organs in the peritoneal cavity, such as the colon. In primary peritoneal cancer, cancer is found in the pelvic peritoneum and has not spread there from another part of the body.
In stage II, cancer is found in one or both ovaries or fallopian tubes and has spread into other areas of the pelvis, or primary peritoneal cancer is found within the pelvis.
Stage II is subdivided based on whether the cancer has spread to other tissue.
Stage IIA: The cancer has spread from where it first formed to the uterus and/or the fallopian tubes and/or the ovaries.
Stage IIB: The cancer has spread from the ovary or fallopian tube to organs in the peritoneal cavity (the body cavity that contains most of the organs in the abdomen).
Stage III (also called stage 3) ovarian cancer
EnlargeTumor sizes are often measured in centimeters (cm) or inches. Common food items that can be used to show tumor size in cm include: a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm or 2 inches), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm or 4 inches).
In stage III, cancer is found in one or both ovaries or fallopian tubes, or is primary peritoneal cancer, and has spread outside the pelvis to other parts of the abdomen and/or to nearby lymph nodes.
Stage III is subdivided based on how far the cancer has spread.
Stage IIIA1: The cancer has spread to lymph nodes behind the peritoneum only.
Stage IIIA2: The cancer cells that can be seen only with a microscope have spread to the surface of the peritoneum outside the pelvis, such as the omentum (a fold of the peritoneum that surrounds the stomach and other organs in the abdomen). Cancer may have spread to nearby lymph nodes. EnlargeIn stage IIIA, cancer is found in one or both ovaries or fallopian tubes and (a) cancer has spread to lymph nodes behind the peritoneum only, or (b) cancer cells that can be seen only with a microscope have spread to the surface of the peritoneum outside the pelvis, such as the omentum. Cancer may have also spread to nearby lymph nodes.
Stage IIIB: The cancer has spread to the peritoneum outside the pelvis, such as the omentum, and the cancer in the peritoneum is 2 centimeters or smaller. Cancer may have spread to lymph nodes behind the peritoneum. EnlargeIn stage IIIB, cancer is found in one or both ovaries or fallopian tubes and has spread to the peritoneum outside the pelvis, such as the omentum. The cancer in the omentum is 2 centimeters or smaller. Cancer may have also spread to lymph nodes behind the peritoneum.
Stage IIIC: The cancer has spread to the peritoneum outside the pelvis, such as the omentum, and the cancer in the peritoneum is larger than 2 centimeters. Cancer may have spread to lymph nodes behind the peritoneum or to the surface of the liver or spleen. EnlargeIn stage IIIC, cancer is found in one or both ovaries or fallopian tubes and has spread to the peritoneum outside the pelvis, such as the omentum. The cancer in the omentum is larger than 2 centimeters. Cancer may have also spread to lymph nodes behind the peritoneum or to the surface of the liver or spleen (not shown).
Stage IV (also called stage 4) ovarian cancer
EnlargeIn stage IV, cancer has spread beyond the abdomen to other parts of the body. In stage IVA, cancer cells are found in extra fluid that builds up around the lungs. In stage IVB, cancer has spread to organs and tissues outside the abdomen, including the lung, liver, and lymph nodes in the groin.
In stage IV, cancer has spread beyond the abdomen to other parts of the body. Stage IV is divided into stage IVA and stage IVB.
Stage IVA: Cancer cells are found in extra fluid that builds up around the lungs.
Stage IVB: Cancer has spread to organs and tissues outside the abdomen, including lymph nodes in the groin.
Stage IV ovarian cancer is also called metastatic ovarian cancer. Metastatic cancer happens when cancer cells travel through the lymphatic system or blood and form tumors in other parts of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if ovarian cancer spreads to the lung, the cancer cells in the lung are actually ovarian cancer cells. The disease is called metastatic ovarian cancer, not lung cancer. Learn more about Metastatic Cancer: When Cancer Spreads.
Recurrent ovarian cancer
Recurrent ovarian cancer is cancer that has come back after it has been treated. The cancer may come back in the ovary or as metastatic tumors in other parts of the body. Tests will be done to help determine where the cancer has returned in the body, if it has spread, and how far. The type of treatment that your child will have for recurrent ovarian cancer will depend on how far it has spread.
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for childhood ovarian cancer. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:
There are different types of treatment for children and adolescents with ovarian tumors. You and your child’s cancer care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health, whether the tumor is benign or cancerous, and whether the tumor is newly diagnosed or has come back.
Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s cancer care team before treatment begins about what to expect. For help every step of the way, see our booklet, Children with Cancer: A Guide for Parents.
The types of treatment your child might have include:
Surgery
Surgery is used to remove a tumor in the ovary. Sometimes the ovary and/or fallopian tube are also removed. During surgery to remove the tumor, fluid in the abdomen will be checked for signs of cancer.
Chemotherapy
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing.
Chemotherapy may be given in different ways. For ovarian cancer, chemotherapy is injected into a vein. When given this way, the drugs enter the bloodstream and can affect cancer cells throughout the body. Chemotherapy drugs used alone or in combination to treat ovarian cancer in children include:
Other chemotherapy drugs not listed here may also be used.
Learn more about how chemotherapy works, how it is given, common side effects, and more at Chemotherapy to Treat Cancer.
Stem cell transplant
High doses of chemotherapy are given to kill cancer cells. This cancer treatment destroys healthy cells, including blood-forming cells. Stem cell transplant (also called stem cell rescue) is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, 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.
Targeted therapy
Targeted therapy uses drugs or other substances to block the action of specific enzymes, proteins, or other molecules involved in the growth and spread of cancer cells.
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
chemotherapy for cancer that has recurred (come back)
Treatment of childhood small cell carcinoma of the ovary
Treatment of newly diagnosed small cell carcinoma of the ovary in children may include:
surgery followed by chemotherapy and high-dose chemotherapy with stem cell rescue
targeted therapy (tazemetostat)
Treatment of recurrent ovarian cancer
If the cancer comes back after treatment, your child’s doctor will talk with you about what to expect and possible next steps. There might be treatment options that may shrink the cancer or control its growth. If there are no treatment options, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
Side effects and late effects of treatment
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Physical problems, such as problems with fertility, are a late effect of treatment.
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. Learn more about Late Effects of Treatment for Childhood Cancer.
Follow-up care
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
Coping with your child's cancer
When a child has a tumor, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, visit Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.
Related resources
For more childhood cancer information and other general cancer resources, visit:
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Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood ovarian cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
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Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
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Testicular cancer most often begins in germ cells (cells that make sperm). It is rare and is most frequently diagnosed in men ages 20 to 34. Most testicular cancers can be cured, even if diagnosed at an advanced stage. Explore the links on this page to learn more about testicular cancer screening, treatment, statistics, and clinical trials.
This summary is about germ cell tumors that form in parts of the body that are extracranial (outside the brain). Extracranial germ cell tumors usually form in the following areas of the body:
EnlargeExtracranial germ cell tumors form in parts of the body other than the brain. This includes the testicles, ovaries, sacrum (lower part of the spine), coccyx (tailbone), mediastinum (area between the lungs), retroperitoneum (the back wall of the abdomen), and the head and neck.
Extracranial germ cell tumors are most common in adolescents, with rates in this age group lower for females than males.
Childhood extracranial germ cell tumors are grouped as gonadal or extragonadal extracranial tumors.
Malignant extracranial germ cell tumors are tumors that form outside the brain. They are gonadal or extragonadal.
Gonadal germ cell tumors
Gonadal germ cell tumors form in the gonads (testicles and ovaries).
Testicular germ cell tumors.Testicular germ cell tumors are divided into two main types, seminoma and nonseminoma. Nonseminomas are usually large and cause signs or symptoms of disease. They tend to grow and spread more quickly than seminomas.
Testicular germ cell tumors usually occur before the age of 5 years or in adolescents and young adults. Testicular germ cell tumors in adolescents (11 years and older) and young adults are different from those that form in early childhood.
Most extragonadal extracranial germ cell tumors 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 (tailbone).
Mediastinum (the area between the lungs).
Back of the abdomen.
Neck.
In children younger than 11 years, extragonadal extracranial germ cell tumors usually occur at birth or in early childhood. Most of these tumors are benign teratomas in the sacrum or coccyx.
In older children, adolescents, and young adults (11 years and older), extragonadal extracranial germ cell tumors are often in the mediastinum.
There are three types of extracranial germ cell tumors.
Extracranial germ cell tumors are also grouped into teratomas, malignant germ cell tumors, and mixed germ cell tumors:
Teratomas
There are two main types of teratomas:
Mature teratomas. These tumors are the most common type of extracranial germ cell tumor. Mature teratomas are benign tumors and not likely to become cancer. They usually occur in the sacrum or coccyx in newborns or in the testicles or ovaries at the start of puberty. The cells of mature teratomas look almost like normal cells under a microscope. Some mature teratomas release enzymes or hormones that cause signs and symptoms of disease.
Immature teratomas. These tumors usually occur in areas other than the gonads in young children or in the ovaries at the start of puberty. They have cells that look very different from normal cells under a microscope. Immature teratomas may be cancer and spread to other parts of the body. They often have several different types of tissue in them, such as hair, muscle, and bone. Some immature teratomas release enzymes or hormones that cause signs and symptoms of disease.
Malignant germ cell tumors
Malignant germ cell tumors are cancer. There are two main types of malignant germ cell tumors:
Seminomatous germ cell tumors. There are three types of seminomatous germ cell tumors:
Mixed germ cell tumors are made up of at least two types of malignant germ cell tumor. They can form in the ovary, testicle, or other areas of the body.
The cause of most childhood extracranial germ cell tumors is unknown.
Having certain inherited disorders can increase the risk of extracranial germ cell tumors.
A risk factor is anything that increases the chance of getting a disease. Not every child with one or more of these risk factors will develop an extracranial germ cell tumor. And it will develop in some children who don’t have a known risk factor.
Possible risk factors for extracranial germ cell tumors include:
Having an ovary or testicle that has not formed normally (gonadal dysgenesis) may increase the risk of gonadoblastoma.
Talk with your child’s doctor if you think your child may be at risk.
Signs of childhood extracranial germ cell tumors depend on where the tumor formed in the body.
Different tumors may cause the following signs and symptoms. Other conditions may cause these same signs and symptoms. It’s important to check with your child’s doctor if your child has:
Imaging studies and blood tests are used to diagnose childhood extracranial germ cell tumors.
If your child has symptoms that suggest they have an extracranial germ cell tumor, their doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with an extracranial germ cell tumor, the results of these tests will help you and your child’s doctor plan treatment.
The tests used to diagnose extracranial germ cell tumors may include:
Serum tumor marker test: A procedure in which a sample of blood is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers.
Some malignant germ cell tumors release tumor markers. The following tumor markers may be used to detect extracranial germ cell tumors:
Alpha-fetoprotein (AFP).
Beta-human chorionic gonadotropin (beta-hCG).
For testicular germ cell tumors, blood levels of the tumor markers help show if the tumor is a seminoma or nonseminoma.
Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body, including lactate dehydrogenase. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
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.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or lymph nodes, 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, computerized tomography, or computerized axial tomography. EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). EnlargeMagnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. EnlargeAbdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Sometimes an incisional biopsy or needle biopsy is done before surgery to remove a sample of tissue. Sometimes the tumor is removed during surgery and then a sample of tissue is removed from the tumor.
The following tests may be done on the sample of tissue that is removed:
Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a sample of tissue are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in certain chromosomes may be a sign of cancer. Cytogenetic analysis is used to help diagnose cancer, plan treatment, or find out how well treatment is working.
Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis and treatment options depend on the following:
The child’s age and general health.
The stage of the cancer (whether it has spread to nearby areas, lymph nodes, or to other places in the body).
Whether the tumor can be completely removed by surgery.
Whether the cancer has just been diagnosed or has recurred (come back).
The prognosis for childhood extracranial germ cell tumors, especially ovarian germ cell tumors, is good.
Stages of Childhood Extracranial Germ Cell Tumors
Key Points
After a childhood extracranial germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread from where the tumor started to nearby areas or to other parts of the body.
There are three ways that cancer spreads in the body.
Cancer may spread from where it began to other parts of the body.
Stages are used to describe the different types of extracranial germ cell tumors.
Testicular germ cell tumors in children younger than 11 years
Testicular germ cell tumors in adolescents and young adults 11 years and older
Ovarian germ cell tumors
Extragonadal extracranial germ cell tumors
Childhood extracranial germ cell tumors usually do not come back after treatment.
After a childhood extracranial germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread from where the tumor started to nearby areas or to other parts of the body.
The process used to find out if cancer has spread from where the tumor started to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. In some cases, staging may follow surgery to remove the tumor.
The following procedures may be used:
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain or lymph nodes. This procedure is also called nuclear magnetic resonance imaging (NMRI).
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or lymph nodes, 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, computerized tomography, or computerized axial tomography.
Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.
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.
Tissue. The cancer spreads from where it began by growing into nearby areas.
Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.
Cancer may spread from where it began to other parts of the body.
When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.
Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if an extracranial germ cell tumor spreads to the liver, the cancer cells in the liver are actually cancerous germ cells. The disease is metastatic extracranial germ cell tumor, not liver cancer.
Many cancer deaths are caused when cancer moves from the original tumor and spreads to other tissues and organs. This is called metastatic cancer. This animation shows how cancer cells travel from the place in the body where they first formed to other parts of the body.
Stages are used to describe the different types of extracranial germ cell tumors.
Testicular germ cell tumors in children younger than 11 years
The following stages are from the Children’s Oncology Group.
Stage I
In stage I, the cancer is found in the testicle only. The testicle and spermatic cord are completely removed by surgery and all of the following are true:
the capsule (outer covering of the tumor) did not rupture (break open) and a biopsy was not done before the tumor was removed; and
the capsule (outer covering of the tumor) ruptured (broke open) or a biopsy was done before surgery; or
cancer that can only be seen with a microscope remains in the scrotum or in the spermatic cord near the scrotum, and after surgery, tumor marker levels do not return to normal or do not decrease.
lymph nodes are at least 2 centimeters wide or are larger than 1 centimeter but smaller than 2 centimeters in their shortest diameter and either have not changed or are growing when a CT scan or MRI is repeated within 4 to 6 weeks.
Stage IV
In stage IV, the cancer has spread to other parts of the body, such as the liver, lung, bone, and brain.
Testicular germ cell tumors in adolescents and young adults 11 years and older
In males older than 15 years, there are only stage I tumors and metastatic tumors. For more information about staging used for testicular germ cell tumors in adolescents and young adults 11 years and older, see Testicular Cancer Treatment.
Ovarian germ cell tumors
Two staging systems are used for ovarian germ cell tumors: Children’s Oncology Group and the International Federation of Gynecology and Obstetrics (FIGO).
The following stages are from the Children’s Oncology Group.
Stage I
In stage I, the tumor in the ovary is completely removed by surgery and all of the following are true:
the capsule (outer covering of the tumor) did not rupture (break open) and a biopsy was not done before the tumor was removed; and
there is no sign that the cancer has spread through the capsule; and
no cancer is seen in tissue that lines the abdomen or found in tissue samples taken during a biopsy; and
lymph nodes are smaller than 1 centimeter in their shortest diameter on a CT scan or MRI or no cancer is found in lymph node tissue samples taken during a biopsy.
Stage II
In stage II, the tumor in the ovary is completely removed by surgery and a biopsy is done before surgery and one of the following is true:
cancer has spread through part or all of the capsule (outer covering of the tumor); or
the tumor is removed by being broken up into small pieces and it is not known if cancer has spread through the capsule.
Cancer cells are not found in fluid taken from the abdomen. Cancer is not seen in lymph nodes or tissue that lines the abdomen and cancer is not found in tissue samples taken during a biopsy.
Stage III
In stage III, there is tumor in the ovary and one of the following is true:
lymph nodes are at least 2 centimeters wide or are larger than 1 centimeter but smaller than 2 centimeters in their shortest diameter and either have not changed or are growing when a CT scan or MRI is repeated 4 to 6 weeks after surgery; or
the tumor is not completely removed by surgery or a biopsy was done before surgery; or
In stage II, cancer is found in one or both ovaries and has spread into other areas of the pelvis, or primary peritoneal cancer is found. Stage II is divided into stage IIA and stage IIB.
Stage IIA: Cancer has spread to the uterus and/or the fallopian tubes (the long slender tubes through which eggs pass from the ovaries to the uterus).
In stage III, cancer is found in one or both ovaries or primary peritoneal cancer is found. Cancer has spread outside the pelvis to other parts of the abdomen and/or to lymph nodes at the back of the abdomen. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC.
EnlargeTumor sizes are often measured in centimeters (cm) or inches. Common food items that can be used to show tumor size in cm include: a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm or 2 inches), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm or 4 inches).
In stage IIIA, one of the following is true:
cancer has spread to lymph nodes at the back of the abdomen only; or
cancer cells that can be seen only with a microscope have spread to the surface of the peritoneum outside the pelvis. Cancer may have spread to nearby lymph nodes at the back of the abdomen.
Stage IIIB: Cancer has spread to the peritoneum outside the pelvis and the cancer in the peritoneum is 2 centimeters or smaller. Cancer may have spread to lymph nodes at the back of the abdomen.
Stage IIIC: Cancer has spread to the peritoneum outside the pelvis and the cancer in the peritoneum is larger than 2 centimeters. Cancer may have spread to lymph nodes at the back of the abdomen or to the surface of the liver or spleen.
cancer is not completely removed by surgery and cancer that can be seen with the eye remains after surgery or only a biopsy was done; or
lymph nodes are at least 2 centimeters wide or are larger than 1 centimeter but smaller than 2 centimeters in their shortest diameter and either have not changed or are growing when a CT scan or MRI is repeated within 4 to 6 weeks.
Stage IV
In stage IV, the cancer has spread to other parts of the body, such as the liver, lung, bone, or brain.
Childhood extracranial germ cell tumors usually do not come back after treatment.
Recurrent childhood extracranial germ cell tumor is cancer that has come back after it has been treated. The cancer may come back in the same place or in other parts of the body.
Most germ cell tumors do not recur (come back) after treatment. If they do recur, they usually come back within 3 years of surgery. About half of the teratomas that recur in the sacrum or coccyx are cancer, so follow-up is important.
Treatment Option Overview
Key Points
There are different types of treatment for children with extracranial germ cell tumors.
Children with extracranial germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
The following types of treatment are used:
Surgery
Observation
Chemotherapy
New types of treatment are being tested in clinical trials.
High-dose chemotherapy with stem cell transplant
Radiation therapy
Treatment for childhood extracranial germ cell tumors may cause side effects.
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
There are different types of treatment for children with extracranial germ cell tumors.
Different types of treatments are available for children with extracranial germ cell tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. 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.
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.
Children with extracranial germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with extracranial germ cell tumors and who specialize in certain areas of medicine. These may include the following specialists:
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. A goal of surgery is to keep reproductive function. The following types of surgery may be used:
Resection: Surgery to remove tissue or part or all of an organ.
After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy after surgery to kill any cancer cells that are left. Treatment given after surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
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). Systemic chemotherapy is used to treat extracranial germ cell tumors.
New types of treatment are being tested in clinical trials.
High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood-forming cells, are also destroyed by the cancer treatment. Stem cell transplant is a treatment to replace the blood-forming cells. 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 patient completes chemotherapy, 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.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. External radiation therapy is being studied for the treatment of childhood extracranial germ cell tumors that have come back.
Treatment for childhood extracranial germ cell tumors may cause side effects.
Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
Physical problems, such as infertility, trouble hearing and kidney problems.
Changes in mood, feelings, thinking, learning, or memory.
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the effects cancer treatment can have on your child. For more information, see Late Effects of Treatment for Childhood Cancer.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
For childhood extracranial germ cell tumors, follow-up may include regular physical exams, tumor marker tests, and imaging tests such as CT scan, MRI or chest x-ray.
Treatment of Childhood Mature and Immature Teratomas
Surgery to remove the tumor followed by observation for stage I tumors.
Surgery to remove the tumor for stage I–IV tumors. In young children, surgery is followed by observation; the use of chemotherapy after surgery is controversial. In adolescents and young adults, chemotherapy is given after surgery.
Sometimes a mature or immature teratoma also has malignantcells. A teratoma with malignant cells may need to be treated differently.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of Malignant Testicular Germ Cell Tumors
Surgery (radical inguinal orchiectomy) followed by chemotherapy for stage II–IV tumors and repeat surgery to remove any remaining tumor.
A clinical trial of a new regimen of surgery followed by observation for stage I tumors or chemotherapy for stage II–IV tumors.
For boys 11 years and older:
Malignant testicular germ cell tumors in boys 11 years and older are treated differently than they are in young boys. For more information, see Testicular Cancer Treatment.
Surgery to remove the tumor. Sometimes lymph nodes in the abdomen are also removed.
A clinical trial of a new regimen of surgery followed by observation for stage I tumors or chemotherapy for stage II–IV tumors.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Surgery followed by chemotherapy for stage I–IV tumors.
A clinical trial of a new regimen of surgery followed by observation for stage I tumors or chemotherapy for stage II–IV.
Treatment of newly diagnosed nongerminomas of the ovary in adolescents and young women may include the following:
Surgery and chemotherapy for stage I–IV tumors.
A clinical trial of a new regimen of surgery followed by observation for stage I or chemotherapy for stage II–IV.
Treatment of newly diagnosed nongerminomas of the ovary that cannot be removed by primary surgery without risk to nearby tissue may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of Malignant Extragonadal Extracranial Germ Cell Tumors
Biopsy followed by chemotherapy with or without surgery for stage III and IV tumors.
In addition to stage of the disease, treatment of malignant extragonadal extracranial germ cell tumors also depends on where the tumor formed in the body:
For tumors in the sacrum or coccyx, chemotherapy to shrink the tumor followed by surgery to remove the tumor and coccyx.
For tumors in the mediastinum, chemotherapy before or after surgery to remove the tumor in the mediastinum.
For tumors in the abdomen, biopsy followed by chemotherapy to shrink the tumor and surgery to remove the tumor in the abdomen.
For tumors in the head and neck, surgery to remove the tumor in the head or neck, which may be followed by chemotherapy if the tumor is cancer.
Treatment of newly diagnosed childhood malignant extragonadal extracranial germ cell tumors in adolescents and young adults may include the following:
Surgery.
Chemotherapy.
Chemotherapy followed by surgery to remove the tumor.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of Recurrent Childhood Malignant Extracranial Germ Cell Tumors
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
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Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood extracranial germ cell tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
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The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become “standard.” Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI’s website. For more information, call the Cancer Information Service (CIS), NCI’s contact center, at 1-800-4-CANCER (1-800-422-6237).
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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Extracranial Germ Cell Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/extracranial-germ-cell/patient/germ-cell-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389180]
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Childhood Testicular Cancer (PDQ®)–Patient Version
What is childhood testicular cancer?
Testicular tumors in children can form in the tissue of one or both testicles. These tumors can be benign (not cancer) or malignant (cancerous). Benign tumors do not spread to other parts of the body. However, they may grow and press on nearby tissues, causing discomfort or other problems. Malignant tumors may spread to other parts of the body. Benign tumors are more common in young boys and infants while malignant tumors are more common in boys who have gone through puberty.
The testicles are two egg-shaped glands in the male reproductive system. They are located inside the scrotum, a sac of loose skin that lies directly below the penis. The testicles are held within the scrotum by the spermatic cord, which contains the vas deferens, vessels, and nerves of the testicles. The testicles produce male hormones, such as testosterone, and sperm.
EnlargeAnatomy of the male reproductive system. The male reproductive system is made up of organs and glands involved in making offspring (children). These include the vas deferens (a long tube that carries sperm out of the testes), prostate gland, penis, and testes.
Types of testicular tumors
There are two types of testicular tumors.
Non-germ cell tumors begin in the cells that produce hormones and in the tissue that surrounds and supports the testicles. These tumors may be benign or cancerous. Types of non-germ cell tumors include:
Juvenile granulosa cell tumors, which most often occur in young infants.
Sertoli cell tumors, which often occur in older infants.
Leydig cell tumors, which are more common in adolescents.
Germ cell tumors begin in the sperm-producing cells of the testicles. Most childhood testicular tumors are germ cell tumors. Learn more about testicular germ cell tumors and their treatment at Childhood Extracranial Germ Cell Tumors Treatment.
Causes and risk factors for childhood testicular cancer
Childhood testicular cancer is caused by certain changes to the way testicular cells function, especially how they grow and divide into new cells. Often, the exact cause of these cell changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Not every child with one or more of these risk factors will develop testicular cancer. And it will develop in some children who don’t have a known risk factor.
The risk of testicular cancer may be increased if your child has one of the following syndromes:
DICER1 syndrome is a disorder that may cause a goiter, polyps in the colon, and tumors of the ovary, cervix, testicle, kidney, brain, eye, and lining of the lung.
Peutz-Jeghers syndrome is a disorder that causes polyps to form in the intestines and dark spots to form on the mouth and fingers.
Carney syndrome or complex is a disorder that causes dark spots on the skin and tumors to form in the heart, endocrine glands, skin, and nerves.
Talk with your child’s doctor if you think your child may be at risk.
Genetic counseling for children with testicular cancer
It may not be clear from the family medical history whether a child with testicular cancer has an inherited condition that increased their risk. Genetic counseling before genetic testing can help assess your child’s risk of having a gene change that caused your child’s tumor and whether genetic testing is needed. Genetic testing may help explain why a child develops a rare cancer or a cancer that is usually seen in adults, such as testicular cancer. Genetic counselors and other specially trained health professionals can discuss your child’s diagnosis and your family’s medical history to help you understand:
the options for DICER1 gene testing
the risk of other cancers for your child
the risk of testicular cancer and other cancers for your child’s siblings
the risks and benefits of learning genetic information
Genetic counselors can also help you cope with your child’s genetic testing results, including how to discuss the results with family members. They can advise you about whether other members of your family should receive genetic testing.
Children may not have symptoms of testicular cancer until the tumor has grown bigger. It’s important to check with your child’s doctor if your child has:
These symptoms may be caused by problems other than childhood testicular cancer. The only way to know is to see your child’s doctor.
Tests to diagnose childhood testicular cancer
If your child has symptoms that suggest testicular cancer, the doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child’s personal and family medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with testicular cancer, the results of these tests will help you and your child’s doctor plan treatment.
The tests and procedures used to diagnose testicular cancer may include:
Ultrasound exam
An ultrasound exam uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
CT scan (CAT scan)
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the chest, abdomen, or pelvis. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. 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, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the chest, abdomen, or pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).
EnlargeMagnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.
Chest x-ray
An x-ray is a type of radiation that can go through the body and make pictures. A chest x-ray makes pictures of the organs and bones inside the chest.
Biopsy
A biopsy is a procedure in which a sample of tissue is removed from the tumor so that a pathologist can view it under a microscope to check for signs of cancer.
Serum tumor marker test
A serum tumor marker test examines a sample of blood to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. If an increased level of the tumor marker alpha-fetoprotein is found in the blood, it means the patient has a testicular germ cell tumor, not a testicular non-germ cell tumor.
DICER1 genetic testing
DICER1 genetic testing examines a sample of blood or saliva for changes in the DICER1 gene.
Getting a second opinion
You may want to get a second opinion to confirm your child’s testicular cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the genetic test results, pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s cancer.
To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, see Questions to Ask Your Doctor about Cancer.
Who treats children with testicular cancer?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for testicular cancer. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:
There are different types of treatment for children and adolescents with testicular cancer. You and your child’s cancer care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the cancer is newly diagnosed or has come back.
Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s cancer care team before treatment begins about what to expect. For help every step of the way, see our booklet, Children with Cancer: A Guide for Parents.
Treatment for non-germ cell testicular cancer in children is usually surgery to remove the tumor. In some cases, the entire testicle with cancer may need to be removed.
If the cancer comes back after treatment, your child’s doctor will talk with you about what to expect and possible next steps. There may be treatment options to shrink the cancer or control its growth. If there are no treatment options, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
Clinical trials
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
If your child has been diagnosed with testicular cancer, you likely have questions about how serious the cancer is and your child’s chances of survival. The likely outcome or course of a disease is called prognosis. In children, the prognosis for testicular cancer is usually excellent after surgery to remove the tumor.
Side effects and late effects of treatment
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child’s treatment team about which side effects to look for and ways to manage them.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Physical problems, such as problems with fertility, are a late effect of treatment.
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. Learn more about Late Effects of Treatment for Childhood Cancer.
Follow-up care
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child’s condition has changed or if the cancer has recurred (come back).
Coping with your child's cancer
When a child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, see Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.
Related resources
For more childhood cancer information and other general cancer resources, see:
Physician Data Query (PDQ) is the National Cancer Institute’s (NCI’s) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
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Childhood bladder cancer is a very rare type of cancer that forms in the tissues of the bladder.
The bladder is a hollow, balloon-shaped organ in the lower part of the abdomen that stores urine. The bladder has a muscular wall that allows it to get larger to store urine made by the kidneys, and to shrink to squeeze urine out of the body. There are two kidneys, one on each side of the backbone, above the waist. The bladder and kidneys work together to remove toxins and wastes from the body through urine:
Tiny tubules in the kidneys filter and clean the blood.
These tubules take out waste products and make urine.
The urine passes from each kidney through a long tube called a ureter into the bladder.
The bladder holds the urine until it passes through the urethra and leaves the body.
EnlargeAnatomy of the urinary system showing the kidneys, ureters, bladder, and urethra. The inside of the left kidney shows the renal pelvis. An inset shows the renal tubules and urine. Also shown is the spine and adrenal glands. Urine is made in the renal tubules and collects in the renal pelvis of each kidney. The urine flows from the kidneys through the ureters to the bladder. The urine is stored in the bladder until it leaves the body through the urethra.
Urothelial carcinoma (also called transitional cell carcinoma) is cancer that begins in the urothelial cells, which line the urethra, bladder, ureters, renal pelvis, and some other organs. The cells of the urothelium are known as transitional cells because they are able to stretch when the bladder is full of urine and shrink when it is emptied. Urothelial carcinoma is the most common form of bladder cancer in children.
Squamous cell and other more aggressive types of bladder cancer are less common in children.
Bladder cancer can occur at any age and most often affects males.
Risk factors for childhood bladder cancer
Bladder cancer is caused by certain changes to the way bladder cells function, especially how they grow and divide into new cells. Learn more about how cancer develops at What Is Cancer?
The risk of bladder cancer is increased in children who have been treated for cancer with certain anticancer drugs, called alkylating agents, which include cyclophosphamide, ifosfamide, busulfan, and temozolomide. Survivors of heritableretinoblastoma also have an increased risk of developing bladder cancer. Not every child with these risk factors will develop bladder cancer. And it will develop in some children who don’t have a known risk factor. Talk with your child’s doctor if you’re concerned about your child’s risk.
Symptoms of childhood bladder cancer
The symptoms of bladder cancer can vary from person to person. The most common symptom of bladder cancer is blood in the urine, called hematuria. It’s often slightly rusty to bright red in color. You may see blood in your child’s urine at one point, then not see it again for a while. Sometimes there are very small amounts of blood in the urine that can only be found by having a test done.
Other common symptoms of bladder cancer can include:
frequent urination or feeling the need to urinate without being able to do so
pain during urination
abdominal or lower back pain
It’s important to check with your child’s doctor if your child has any of these symptoms. Keep in mind that urinary tract infections, kidney or bladder stones, or other problems related to the kidney could also be the cause, not cancer. Your child’s doctor will ask you when the symptoms started and how often your child has been having them. They’ll most likely ask for a urine sample as a first step in diagnosing what is causing these symptoms.
Tests to diagnose childhood bladder cancer
If your child has symptoms or lab test results that suggest bladder cancer, their doctor will need to find out if these are due to cancer or another problem. They may:
ask about your child’s personal and family medical history to learn more about your child’s symptoms and possible risk factors for bladder cancer
do a physical exam to check for signs of cancer
ask for a sample of urine so it can be checked in the lab for blood, abnormal cells, or infection
Depending on your child’s symptoms and medical history and the results of their physical exam and urine lab tests, the doctor may recommend more tests to find out if your child has bladder cancer, and if so, its extent (stage). The results of tests and procedures done to diagnose bladder cancer are used to help make decisions about treatment.
The following tests and procedures may be used:
CT scan (CAT scan)
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the pelvis. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Learn more about Computed Tomography (CT) Scans and Cancer.
EnlargeComputed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
Ultrasound exam
An ultrasound exam uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs, such as the pelvis, and make echoes. The echoes form a picture of body tissues called a sonogram.
EnlargeAbdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
Cystoscopy
Cystoscopy is a procedure in which the doctor looks inside the bladder and urethra (the tube that carries urine out of your body) to check for abnormal areas. A cystoscope is slowly inserted through the urethra into the bladder to allow the doctor to see inside. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove very small tumors or tissue samples for biopsy. If a cystoscopy is not done at diagnosis, tissue samples are removed and checked for cancer during surgery to remove all or part of the bladder.
Getting a second opinion
You may want to get a second opinion to confirm your child’s bladder cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. They may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child’s tumor.
To learn more about choosing a doctor and getting a second opinion, see Finding Cancer Care. You can contact NCI’s Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child’s appointments, see Questions to Ask Your Doctor about Cancer.
Prognosis of childhood bladder cancer
If your child has been diagnosed with bladder cancer, you likely have questions about how serious the cancer is and your child’s chances of survival. The likely outcome or course of a disease is called prognosis.
The prognosis can be affected by whether the cancer can be removed by surgery. In children, bladder cancer is usually low grade (not likely to spread) and the prognosis is usually excellent after surgery to remove the tumor.
Types of treatment for childhood bladder cancer
There are different types of treatment for children and adolescents with bladder cancer. You and your child’s cancer care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the cancer is newly diagnosed or has come back.
A pediatric oncologist, a doctor who specializes in treating children with cancer, will oversee treatment for childhood bladder cancer. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. Other specialists may include:
Your child’s treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s cancer care team before treatment begins about what to expect. For help every step of the way, see our downloadable booklet, Children with Cancer: A Guide for Parents.
Types of treatment your child might have include:
Surgery
Surgery to remove the cancer and part or all of the bladder is the standard treatment for bladder cancer in children. The type of surgery depends on where the cancer is located and whether it is aggressive.
During TUR, the doctor removes tissue from the bladder using a resectoscope inserted into the bladder through the urethra. A resectoscope is a thin, tube-like instrument with a light, a lens for viewing, and a tool to remove tissue and burn away any remaining tumor cells. Tissue samples from the area where the tumor was removed are checked under a microscope for signs of cancer.
Cystectomy
Cystectomy, surgery to remove part or all of the bladder, is rarely used to treat bladder cancer in children. However, it may be needed in children with squamous cell carcinoma or more aggressive carcinomas.
Clinical trials
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child’s age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Sometimes bladder cancer can recur (come back) after treatment. If your child is diagnosed with recurrent bladder cancer, your child’s doctor will work with you to plan treatment.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
physical problems
changes in mood, feelings, thinking, learning, or memory
second cancers (new types of cancer) or other conditions
Some late effects may be treated or controlled. It is important to talk with your child’s doctors about the possible late effects caused by some treatments. Learn more about Late Effects of Treatment for Childhood Cancer.
Follow-up care
Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment worked.
If the bladder cancer recurs (comes back) after treatment, it will usually be within 3 years from diagnosis. Your child will receive tests from time to time after surgery to find out if their condition has changed or if the cancer has recurred. These tests are sometimes called follow-up tests or check-ups.
Coping and support
When a child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is also important. Reach out to your child’s treatment team and to people in your family and community for support. To learn more, see Support for Families: Childhood Cancer.
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