Childhood Tracheobronchial Tumors (PDQ®)–Patient Version

Childhood Tracheobronchial Tumors (PDQ®)–Patient Version

What are childhood tracheobronchial tumors?

Tracheobronchial tumors are rare, abnormal growths that form in the windpipe (trachea) or the large airways in the lungs called the bronchi. They can be benign (not cancerous) or malignant (cancerous). Benign tumors are more common in children. If a child has a benign tumor, they may need treatment to prevent the tumor from growing and putting pressure on nearby tissue in the airway. If the tumor is cancerous, the treatment is aimed at killing the cancer cells and keeping them from spreading to other parts of the body.

EnlargeDrawing shows areas of the body where tracheobronchial tumors may form, including the trachea and the bronchi (large airways of the lung).
Tracheobronchial tumors form in the inside lining of the trachea or bronchi (large airways of the lung). Tracheobronchial tumors are rare and can occur in both children and adults.

Several types of tracheobronchial tumors may affect children:

Causes and risk factors for childhood tracheobronchial tumors

Tracheobronchial tumors in children are caused by certain changes to the way cells in the lining of the trachea or large bronchi 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. There are no known risk factors for childhood tracheobronchial tumors.

Symptoms of childhood tracheobronchial tumors

Symptoms of tracheobronchial tumors are a lot like symptoms of asthma, which can make it hard to diagnose the tumor. It’s important to check with your child’s doctor if your child has any symptoms, such as:

  • dry cough
  • wheezing
  • trouble breathing
  • coughing up blood
  • frequent infections in the lung, such as pneumonia
  • feeling very tired
  • loss of appetite or weight loss for no known reason

These symptoms may be caused by problems other than a tracheobronchial tumor. The only way to know is to see your child’s doctor.

Tests to diagnose childhood tracheobronchial tumors

If your child has symptoms that suggest a tracheobronchial tumor, the doctor will need to find out if these are due to cancer or to 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 a tracheobronchial tumor, the results of these tests will help you and your child’s doctor plan treatment.

The tests used to diagnose tracheobronchial tumors in children may include:

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.

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 neck and chest. 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; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
Computed 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.

Bronchoscopy

Bronchoscopy is a procedure to look for abnormal areas inside the trachea and large airways in the lung. 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.

Bronchography

Bronchography is a procedure to look for abnormal areas in the larynx, trachea, and bronchi and to check whether the airways are wider below the level of the tumor. A contrast dye is given through a bronchoscope to coat the airways and make them show up more clearly on x-ray film.

Octreotide scan

An octreotide scan is a type of radionuclide scan used to find tracheobronchial tumors or cancer that has spread to the lymph nodes. A very small amount of radioactive octreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body.

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 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, 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, hospital, or getting 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 tracheobronchial tumors?

A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of tracheobronchial tumors. 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:

Treatment of childhood tracheobronchial tumors

There are different types of treatment for children and adolescents with tracheobronchial tumors. You and your child’s care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the tumor is newly diagnosed or has come back after treatment.

Your child’s treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s 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.

For all tracheobronchial tumors except rhabdomyosarcoma, treatment might include surgery to remove the tumor. The lymph nodes and vessels where cancer has spread are also removed. Sometimes a surgery called a sleeve resection is done.

For rhabdomyosarcoma in the trachea or bronchi, treatment might include:

  • 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 alone or with other types of treatment.
  • Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External beam radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.

Learn more about Childhood Rhabdomyosarcoma Treatment.

For inflammatory myofibroblastic tumors in the trachea or bronchi, in addition to surgery, treatment may include targeted therapy. Targeted therapy uses drugs or other substances to identify and attack specific cancer cells. Crizotinib may be used if the tumor has a certain change in the ALK gene. Learn more about Targeted Therapy to Treat 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 treatments, 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.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

Prognosis and prognostic factors for childhood tracheobronchial tumors

If your child has been diagnosed with a tracheobronchial tumor, you likely have questions about how serious the cancer is and about your child’s chances of survival. The likely outcome or course of a disease is called prognosis.

The prognosis depends on many factors, including:

  • the type of tracheobronchial tumor
  • whether the tumor is or has become cancer and spread to other parts of the body
  • whether the tumor was completely removed by surgery
  • whether the tumor is newly diagnosed or has come back after treatment

The prognosis for children with tracheobronchial tumors that can be removed by surgery is very good. This is the case for most tracheobronchial tumors except rhabdomyosarcoma, which requires more aggressive treatment.

No two people are alike, and responses to treatment can vary greatly. Your child’s cancer care team is in the best position to talk with you about your child’s prognosis.

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.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

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.

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 your 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, see Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.

Related resources

About This PDQ Summary

About PDQ

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 tracheobronchial 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.

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).

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The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Tracheobronchial Tumors. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/lung/patient/child-tracheobronchial-treatment-pdq. Accessed <MM/DD/YYYY>.

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Childhood Rhabdomyosarcoma Treatment (PDQ®)–Patient Version

Childhood Rhabdomyosarcoma Treatment (PDQ®)–Patient Version

General Information About Childhood Rhabdomyosarcoma

Key Points

  • Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue.
  • Certain genetic conditions increase the risk of childhood rhabdomyosarcoma.
  • A sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger.
  • Diagnostic tests and a biopsy are used to diagnose childhood rhabdomyosarcoma.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue.

Rhabdomyosarcoma is a type of sarcoma. Sarcoma is cancer of soft tissue (such as muscle), connective tissue (such as tendon or cartilage), or bone. Rhabdomyosarcoma usually begins in muscles that are attached to bones and that help the body move, but it may begin in many places in the body. Rhabdomyosarcoma is the most common type of soft tissue sarcoma in children.

There are four main types of rhabdomyosarcoma:

  • Embryonal: This type occurs most often in the head and neck area or in the genital or urinary organs, but can occur anywhere in the body. It is the most common type of rhabdomyosarcoma.
  • Alveolar: This type occurs most often in the arms or legs, chest, abdomen, genital organs, or anal area.
  • Spindle cell/sclerosing: The spindle cell type occurs most often in the paratesticular (testis or spermatic cord) area. There are two other spindle cell/sclerosing subtypes. One is more common in infants and is found in the trunk area. The other can affect children, adolescents, and adults. It is often found in the head and neck area, and is more aggressive.
  • Pleomorphic: This is the least common type of rhabdomyosarcoma in children.

See the following PDQ treatment summaries for information about other types of soft tissue sarcoma:

Certain genetic conditions increase the risk of childhood rhabdomyosarcoma.

Anything that increases the risk 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.

Risk factors for rhabdomyosarcoma include having the following inherited diseases:

Children who had a high birth weight or were larger than expected at birth may have an increased risk of embryonal rhabdomyosarcoma.

In most cases, the cause of rhabdomyosarcoma is not known.

A sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger.

Signs and symptoms may be caused by childhood rhabdomyosarcoma or by other conditions. The signs and symptoms that occur depend on where the cancer forms. Check with your child’s doctor if your child has any of the following:

  • A lump or swelling that keeps getting bigger or does not go away. It may be painful.
  • Crossed-eyes or bulging of the eye.
  • Headache.
  • Trouble urinating or having bowel movements.
  • Blood in the urine.
  • Bleeding in the nose, throat, vagina, or rectum.

Diagnostic tests and a biopsy are used to diagnose childhood rhabdomyosarcoma.

The diagnostic tests that are done depend in part on where the cancer forms. 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.
  • X-ray: An x-ray of the organs and bones inside the body, such as 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, abdomen, pelvis, 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; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.
    Computed 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 of the body, such as the skull, brain, and lymph nodes. This procedure is also called nuclear magnetic resonance imaging (NMRI).
    EnlargeMagnetic resonance imaging (MRI) scan; drawing shows a child lying on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body.
    Magnetic 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.
  • 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; drawing shows a child lying on table that slides through the PET scanner.
    Positron 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.
  • 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.
    EnlargeBone scan; drawing shows a child lying on a table that slides under the scanner, a technician operating the scanner, and a computer monitor that will show images made during the scan.
    Bone scan. A small amount of radioactive material is injected into the child’s vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.
  • Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone. Samples are removed from both hipbones. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
    EnlargeBone marrow aspiration and biopsy; drawing shows a child lying face down on a table and a bone marrow needle being inserted into the right hip bone. An inset shows the bone marrow needle being inserted through the skin into the bone marrow of the hip bone.
    Bone marrow aspiration and biopsy. After a small area of skin is numbed, a bone marrow needle is inserted into the child’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.
  • Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs of cancer cells. This procedure is also called an LP or spinal tap.

If these tests show there may be a rhabdomyosarcoma, a biopsy is done. A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Because treatment depends on the type of rhabdomyosarcoma, biopsy samples should be checked by a pathologist who has experience in diagnosing rhabdomyosarcoma.

One of the following types of biopsies may be used:

  • Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.
  • Core needle biopsy: The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI.
  • Open biopsy: The removal of tissue through an incision (cut) made in the skin.
  • Sentinel lymph node biopsy: The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node in a group of lymph nodes to receive lymphatic drainage from the primary tumor. It is the first lymph node the cancer is likely to spread to from the primary tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. Sometimes, a sentinel lymph node is found in more than one group of nodes. Sentinel lymph node biopsy may be used for patients with rhabdomyosarcoma of the limbs or trunk when enlarged lymph nodes are not found with imaging or physical exam.

The following tests may be done on the sample of tissue that is removed:

  • Light microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
  • 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.
  • FISH (fluorescence in situ hybridization): A laboratory test used to look at and count genes or chromosomes in cells and tissues. Pieces of DNA that contain fluorescent dyes are made in the laboratory and added to a sample of a patient’s cells or tissues. When these dyed pieces of DNA attach to certain genes or areas of chromosomes in the sample, they light up when viewed under a fluorescent microscope. The FISH test is used to help diagnose cancer and help plan treatment.
  • Reverse transcription–polymerase chain reaction (RT–PCR) test: A laboratory test in which the amount of a genetic substance called mRNA made by a specific gene is measured. An enzyme called reverse transcriptase is used to convert a specific piece of RNA into a matching piece of DNA, which can be amplified (made in large numbers) by another enzyme called DNA polymerase. The amplified DNA copies help tell whether a specific mRNA is being made by a gene. RT–PCR can be used to check the activation of certain genes that may indicate the presence of cancer cells. This test may be used to look for certain changes in a gene or chromosome, which may help diagnose cancer.
  • 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.

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

The prognosis and treatment options depend on the following:

  • The patient’s age.
  • Where in the body the tumor started.
  • The size of the tumor at the time of diagnosis.
  • Whether the tumor has been completely removed by surgery.
  • The type of rhabdomyosarcoma (embryonal, alveolar, spindle cell/sclerosing, or pleomorphic).
  • Whether there are certain changes in the genes.
  • Whether the tumor had spread to other parts of the body at the time of diagnosis.
  • Whether the tumor was in the lymph nodes at the time of diagnosis.
  • Whether the tumor responds to chemotherapy and/or radiation therapy.

For patients with recurrent cancer, prognosis and treatment also depend on the following:

  • Where in the body the tumor recurred (came back).
  • How much time passed between the end of cancer treatment and when the cancer recurred.
  • Whether the cancer was previously treated with radiation therapy.

Stages of Childhood Rhabdomyosarcoma

Key Points

  • After childhood rhabdomyosarcoma has been diagnosed, treatment is based in part on the stage of the cancer and sometimes it is based on whether all the cancer was removed by surgery.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • Staging of childhood rhabdomyosarcoma is done in three parts.
  • The staging system is based on the size of the tumor, where it is in the body, and whether it has spread to other parts of the body:
    • Stage 1
    • Stage 2
    • Stage 3
    • Stage 4
  • The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery:
    • Group I
    • Group II
    • Group III
    • Group IV
  • The risk group is based on the staging system and the grouping system.
    • Low-risk childhood rhabdomyosarcoma
    • Intermediate-risk childhood rhabdomyosarcoma
    • High-risk childhood rhabdomyosarcoma
  • Sometimes childhood rhabdomyosarcoma continues to grow or comes back after treatment.

After childhood rhabdomyosarcoma has been diagnosed, treatment is based in part on the stage of the cancer and sometimes it is based on whether all the cancer was removed by surgery.

The process used to find out if cancer has spread within the tissue or to other parts of the body is called staging. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic tests to help find out the stage of the disease.

Treatment for childhood rhabdomyosarcoma is based in part on the stage and sometimes on the amount of cancer that remains after surgery to remove the tumor. The pathologist will use a microscope to check the tissues removed during surgery, including tissue samples from the edges of the areas where the cancer was removed and the lymph nodes. This is done to see if all the cancer cells were taken out during the surgery.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • 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 rhabdomyosarcoma spreads to the lung, the cancer cells in the lung are actually rhabdomyosarcoma cells. The disease is metastatic rhabdomyosarcoma, not lung 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.

Staging of childhood rhabdomyosarcoma is done in three parts.

Childhood rhabdomyosarcoma is staged by using three different ways to describe the cancer:

The staging system is based on the size of the tumor, where it is in the body, and whether it has spread to other parts of the body:

Stage 1

In stage 1, the tumor is any size, may have spread to lymph nodes, and is found in only one of the following “favorable” sites:

Rhabdomyosarcoma that forms in a “favorable” site has a better prognosis. If the site where cancer occurs is not one of the favorable sites listed above, it is said to be an “unfavorable” site.

EnlargeDrawing shows different sizes of a tumor in centimeters (cm) compared to the size of a pea (1 cm), a peanut (2 cm), a grape (3 cm), a walnut (4 cm), a lime (5 cm), an egg (6 cm), a peach (7 cm), and a grapefruit (10 cm). Also shown is a 10-cm ruler and a 4-inch ruler.
Tumor 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).

Stage 2

In stage 2, cancer is found in an “unfavorable” site (any one area not described as “favorable” in stage 1). The tumor is no larger than 5 centimeters and has not spread to lymph nodes.

Stage 3

In stage 3, cancer is found in an “unfavorable” site (any one area not described as “favorable” in stage 1) and one of the following is true:

  • The tumor is no larger than 5 centimeters and cancer has spread to nearby lymph nodes.
  • The tumor is larger than 5 centimeters and cancer may have spread to nearby lymph nodes.

Stage 4

In stage 4, the tumor may be any size and cancer may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body, such as the lung, bone marrow, or bone.

The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery:

Group I

Cancer was found only in the place where it started and it was completely removed by surgery. Tissue was taken from the edges of where the tumor was removed. This tissue was checked under a microscope by a pathologist and no cancer cells were found.

Group II

Group II is divided into groups IIA, IIB, and IIC.

  • IIA: Cancer was removed by surgery but cancer cells were seen when the tissue, taken from the edges of where the tumor was removed, was viewed under a microscope by a pathologist.
  • IIB: Cancer had spread to nearby lymph nodes and the cancer and lymph nodes were removed by surgery.
  • IIC: Cancer had spread to nearby lymph nodes, the cancer and lymph nodes were removed by surgery, and at least one of the following is true:
    • Tissue taken from the edges of where the tumor was removed was checked under a microscope by a pathologist and cancer cells were seen.
    • The furthest lymph node from the tumor that was removed was checked under a microscope by a pathologist and cancer cells were seen.

Group III

Cancer was partly removed by biopsy or surgery but there is tumor remaining that can be seen with the eye.

Group IV

Cancer had spread to distant parts of the body when the cancer was diagnosed.

The risk group is based on the staging system and the grouping system.

The risk group describes the chance that rhabdomyosarcoma will recur (come back). Every child treated for rhabdomyosarcoma should receive chemotherapy to decrease the chance cancer will recur. The type of anticancer drug, dose, and the number of treatments given depends on whether the child has low-risk, intermediate-risk, or high-risk rhabdomyosarcoma.

The following risk groups are used:

Low-risk childhood rhabdomyosarcoma

Low-risk childhood rhabdomyosarcoma is one of the following:

Intermediate-risk childhood rhabdomyosarcoma

Intermediate-risk childhood rhabdomyosarcoma is one of the following:

  • An embryonal tumor of any size that is not found in one of the “favorable” sites listed above. There is tumor remaining after surgery, that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes.
  • An alveolar tumor of any size in a “favorable” or “unfavorable” site. There may be tumor remaining after surgery that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes.

High-risk childhood rhabdomyosarcoma

High-risk childhood rhabdomyosarcoma may be the embryonal type or the alveolar type. It may have spread to nearby lymph nodes and has spread to one or more of the following:

Sometimes childhood rhabdomyosarcoma continues to grow or comes back after treatment.

Progressive rhabdomyosarcoma is cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.

Recurrent childhood rhabdomyosarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body, such as the lung, bone, or bone marrow. Less often, rhabdomyosarcoma may come back in the breast in adolescent females or in the liver.

Treatment Option Overview

Key Points

  • There are different types of treatment for patients with childhood rhabdomyosarcoma.
  • Children with rhabdomyosarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
  • Three types of standard treatment are used:
    • Surgery
    • Radiation therapy
    • Chemotherapy
  • New types of treatment are being tested in clinical trials.
    • Immunotherapy
    • Targeted therapy
  • Treatment for childhood rhabdomyosarcoma 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 patients with childhood rhabdomyosarcoma.

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 rhabdomyosarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

Because rhabdomyosarcoma can form in many different parts of the body, many different kinds of treatments are used. 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 rhabdomyosarcoma and who specialize in certain areas of medicine. These may include the following specialists:

Three types of standard treatment are used:

Surgery

Surgery (removing the cancer in an operation) is used to treat childhood rhabdomyosarcoma. A type of surgery called wide local excision is often done. A wide local excision is the removal of tumor and some of the tissue around it, including the lymph nodes. A second surgery may be needed to remove all the cancer. Whether surgery is done and the type of surgery done depends on the following:

  • Where in the body the tumor started.
  • The effect the surgery will have on the way the child will look.
  • The effect the surgery will have on the child’s important body functions.
  • How the tumor responded to chemotherapy or radiation therapy that may have been given first.

In most children with rhabdomyosarcoma, it is not possible to remove all of the tumor by surgery.

Rhabdomyosarcoma can form in many different places in the body and the surgery will be different for each site. Surgery to treat rhabdomyosarcoma of the eye or genital areas is usually a biopsy. Chemotherapy, and sometimes radiation therapy, may be given before surgery to shrink large tumors.

After the doctor removes all the cancer that can be seen at the time of the surgery, patients will be given chemotherapy after surgery to kill any cancer cells that are left. Radiation therapy may also be given. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or stop them from growing. There are two types of radiation therapy:

  • External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of external radiation therapy include the following:
    • Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to nearby healthy tissue.
    • Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles.
    • Volumetrical modulated arc therapy (VMAT): VMAT is type of 3-D radiation therapy that uses a computer to make pictures of the size and shape of the tumor. The radiation machine moves in a circle around the patient once during treatment and sends thin beams of radiation of different intensities (strengths) at the tumor. Treatment with VMAT is delivered faster than treatment with IMRT.
    • Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to place the patient in the same position for each radiation treatment. Once a day for several days, a radiation machine aims a larger than usual dose of radiation directly at the tumor. By having the patient in the same position for each treatment, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.
    • Proton beam radiation therapy: Proton-beam therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. This type of treatment may cause less damage to nearby healthy tissue.
  • Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. It is used to treat cancer in areas such as the vagina, vulva, uterus, bladder, prostate, head, or neck. Internal radiation therapy is also called brachytherapy, internal radiation, implant radiation, or interstitial radiation therapy. This approach involves special technical skills and is offered in only a few medical centers.

The type and amount of radiation therapy and when it is given depends on the age of the child, the type of rhabdomyosarcoma, where in the body the tumor started, how much tumor remained after surgery, and whether there is tumor in the nearby lymph nodes.

External radiation therapy is usually used to treat childhood rhabdomyosarcoma but in certain cases internal radiation therapy is used.

Chemotherapy

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).

Chemotherapy may also be given to shrink the tumor before surgery in order to save as much healthy tissue as possible. This is called neoadjuvant chemotherapy.

Every child treated for rhabdomyosarcoma should receive systemic chemotherapy to decrease the chance the cancer will recur. The type of anticancer drug, dose, and the number of treatments given depends on the age of the child and whether the child has low-risk, intermediate-risk, or high-risk rhabdomyosarcoma.

See Drugs Approved for Rhabdomyosarcoma for more information.

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.

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. There are different types of immunotherapy:

  • Vaccine therapy is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find the tumor and kill it. Vaccine therapy is being studied to treat metastatic rhabdomyosarcoma.
  • Immune checkpoint inhibitor therapy uses the body’s immune system to kill cancer cells. Two types of immune checkpoint inhibitors are being studied in the treatment of childhood rhabdomyosarcoma that has come back after treatment:
    • CTLA-4 is a protein on the surface of T cells that helps keep the body’s immune responses in check. When CTLA-4 attaches to another protein called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow the T cells to kill cancer cells. Ipilimumab is being studied in the treatment of childhood rhabdomyosarcoma that has come back or progressed during treatment.
      EnlargeImmune checkpoint inhibitor; the panel on the left shows the binding of the T-cell receptor (TCR) to antigen and MHC proteins on the antigen-presenting cell (APC) and the binding of CD28 on the T cell to B7-1/B7-2 on the APC. It also shows the binding of B7-1/B7-2 to CTLA-4 on the T cell, which keeps the T cells in the inactive state. The panel on the right shows immune checkpoint inhibitor (anti-CTLA antibody) blocking the binding of B7-1/B7-2 to CTLA-4, which allows the T cells to be active and to kill tumor cells.
      Immune checkpoint inhibitor. Checkpoint proteins, such as B7-1/B7-2 on antigen-presenting cells (APC) and CTLA-4 on T cells, help keep the body’s immune responses in check. When the T-cell receptor (TCR) binds to antigen and major histocompatibility complex (MHC) proteins on the APC and CD28 binds to B7-1/B7-2 on the APC, the T cell can be activated. However, the binding of B7-1/B7-2 to CTLA-4 keeps the T cells in the inactive state so they are not able to kill tumor cells in the body (left panel). Blocking the binding of B7-1/B7-2 to CTLA-4 with an immune checkpoint inhibitor (anti-CTLA-4 antibody) allows the T cells to be active and to kill tumor cells (right panel).
    • PD-1 and PD-L1 inhibitor therapy: PD-1 is a protein on the surface of T cells that helps keep the body’s immune responses in check. PD-L1 is a protein found on some types of cancer cells. When PD-1 attaches to PD-L1, it stops the T cell from killing the cancer cell. PD-1 and PD-L1 inhibitors keep PD-1 and PD-L1 proteins from attaching to each other. This allows the T cells to kill cancer cells. Nivolumab and pembrolizumab are types of PD-1 inhibitors that are being studied in the treatment of childhood rhabdomyosarcoma that has come back or progressed during treatment.
EnlargeImmune checkpoint inhibitor; the panel on the left shows the binding of proteins PD-L1 (on the tumor cell) to PD-1 (on the T cell), which keeps T cells from killing tumor cells in the body. Also shown are a tumor cell antigen and T cell receptor. The panel on the right shows immune checkpoint inhibitors (anti-PD-L1 and anti-PD-1) blocking the binding of PD-L1 to PD-1, which allows the T cells to kill tumor cells.
Immune checkpoint inhibitor. Checkpoint proteins, such as PD-L1 on tumor cells and PD-1 on T cells, help keep immune responses in check. The binding of PD-L1 to PD-1 keeps T cells from killing tumor cells in the body (left panel). Blocking the binding of PD-L1 to PD-1 with an immune checkpoint inhibitor (anti-PD-L1 or anti-PD-1) allows the T cells to kill tumor cells (right panel).
Immunotherapy uses the body’s immune system to fight cancer. This animation explains one type of immunotherapy that uses immune checkpoint inhibitors to treat cancer.

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. There are different types of targeted therapy:

Treatment for childhood rhabdomyosarcoma may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit 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 for rhabdomyosarcoma may include:

  • Physical problems that affect the following:
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).

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 and the types of symptoms to expect after cancer treatment has ended. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)

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).

Treatment of Childhood Rhabdomyosarcoma

The treatment of newly diagnosed childhood rhabdomyosarcoma often includes surgery, radiation therapy, and chemotherapy. The order that these treatments are given depends on where in the body the tumor started, the size of the tumor, the type of tumor, and whether the tumor has spread to lymph nodes or other parts of the body. See the Treatment Option Overview section of this summary for more information about surgery, radiation therapy, and chemotherapy used to treat children with rhabdomyosarcoma.

Rhabdomyosarcoma of the brain and head and neck

  • For tumors of the brain: Treatment may include surgery to remove the tumor, radiation therapy, and chemotherapy.
  • For tumors of the head and neck that are in or near the eye: Treatment may include chemotherapy and radiation therapy. If the tumor remains or comes back after treatment with chemotherapy and radiation therapy, surgery to remove the eye and some tissues around the eye may be needed.
  • For tumors of the head and neck that are near the ear, nose, sinuses, or base of the skull but not in or near the eye: Treatment may include radiation therapy and chemotherapy.
  • For tumors of the head and neck that are not in or near the eye and not near the ear, nose, sinuses, or base of the skull: Treatment may include chemotherapy, radiation therapy, and surgery to remove the tumor.
  • For tumors of the head and neck that cannot be removed by surgery: Treatment may include chemotherapy and radiation therapy including stereotactic body radiation therapy.
  • For tumors of the larynx (voice box): Treatment may include chemotherapy and radiation therapy. Surgery to remove the larynx is usually not done, so that the voice is not harmed.

Rhabdomyosarcoma of the arms or legs

  • Chemotherapy followed by surgery to remove the tumor. If the tumor was not completely removed, a second surgery to remove the tumor may be done. Radiation therapy may also be given.
  • For tumors of the hand or foot, radiation therapy and chemotherapy may be given. The tumor may not be removed because it would affect the function of the hand or foot.
  • Lymph node dissection (one or more lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer).
    • For tumors in the arms, lymph nodes near the tumor and in the armpit area are removed.
    • For tumors in the legs, lymph nodes near the tumor and in the groin area are removed.

Rhabdomyosarcoma of the chest, abdomen, or pelvis

  • For tumors in the chest or abdomen (including the chest wall or abdominal wall): Surgery (wide local excision) may be done. If the tumor is large, chemotherapy and radiation therapy are given to shrink the tumor before surgery.
  • For tumors of the pelvis: Surgery (wide local excision) may be done. If the tumor is large, chemotherapy is given to shrink the tumor before surgery. Radiation therapy may be given after surgery.
  • For tumors of the diaphragm: A biopsy of the tumor is followed by chemotherapy and radiation therapy to shrink the tumor. Surgery may be done later to remove any remaining cancer cells.
  • For tumors of the gallbladder or bile ducts: A biopsy of the tumor is followed by chemotherapy and radiation therapy. Surgery may be done later to remove any remaining cancer cells.
  • For tumors of the muscles or tissues around the anus or between the vulva and the anus or the scrotum and the anus: Surgery may be done to remove as much of the tumor as possible and some nearby lymph nodes, followed by chemotherapy and radiation therapy.

Rhabdomyosarcoma of the kidney

Rhabdomyosarcoma of the bladder or prostate

Rhabdomyosarcoma of the area near the testicles

Rhabdomyosarcoma of the vulva, vagina, uterus, or ovary

Clinical Trials For Childhood Rhabdomyosarcoma

Metastatic rhabdomyosarcoma

Treatment, such as chemotherapy followed by radiation therapy or surgery to remove the tumor, is given to the site where the tumor first formed. If the cancer has spread to the brain, spinal cord, or lungs, radiation therapy may also be given to the sites where the cancer has spread.

The following treatment is being studied for metastatic rhabdomyosarcoma:

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 Progressive or Recurrent Childhood Rhabdomyosarcoma

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment options for progressive or recurrent childhood rhabdomyosarcoma are based on many factors, including where in the body the cancer has come back, what type of treatment the child had before, and the needs of the child.

Treatment of progressive or recurrent rhabdomyosarcoma may include one or more of 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.

To Learn More About Childhood Rhabdomyosarcoma

About This PDQ Summary

About PDQ

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 rhabdomyosarcoma. 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 Rhabdomyosarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389279]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

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Childhood Breast Cancer Treatment (PDQ®)–Patient Version

Childhood Breast Cancer Treatment (PDQ®)–Patient Version

General Information About Childhood Breast Cancer

Key Points

  • Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.
  • Most breast tumors in children are fibroadenomas (not cancer).
  • Radiation therapy to the breast or chest to treat a previous cancer increases the risk of breast cancer.
  • Genetic counseling may be done for patients with breast cancer and a family history of breast cancer.
  • Signs of breast cancer include a lump or thickening in or near the breast.
  • Tests that examine the breast are used to diagnose breast cancer.

Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast.

The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes. Each lobe has many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can make milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts.

Each breast also has blood vessels and lymph vessels. The lymph vessels carry an almost colorless, watery fluid called lymph. Lymph vessels carry lymph between lymph nodes. Lymph nodes are small, bean-shaped structures found throughout the body. They filter lymph and store white blood cells that help fight infection and disease. Groups of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest.

Breast cancer can occur in the breast tissue of both males and females.

EnlargeIllustration of the female breast anatomy. On the left, a front view shows lymph nodes inside the breast going from the breast to the armpit. On the right, a cross-section shows the chest wall, ribs, fatty tissue, lobes, ducts, and lobules. Also shown in both panels are the muscle, nipple, and areola.
The female breast contains lobes, lobules, and ducts that produce and transport milk to the nipple. Fatty tissue gives the breast its shape, while muscles and the chest wall provide support. The lymphatic system, including lymph nodes, filter lymph and store white blood cells that help fight infection and disease.

Breast cancer is the most common cancer among female adolescents and young adults (age 15 to 39 years), but less than 5% of all breast cancers occur in this age group. Breast cancer in adolescents and young adults is often more aggressive and more difficult to treat than in older women, but treatment options are similar.

Most breast tumors in children are fibroadenomas (not cancer).

Fibroadenomas are benign tumors. Other benign breast tumors may include tubular adenomas, phyllodes tumors, and fibroepithelial neoplasms. While most phyllodes tumors are benign, some can be cancer. If a breast tumor grows quickly, is large in size, or there is more than one tumor, a core-needle biopsy or an excisional biopsy will be done. The tissues removed during the biopsy will be viewed under a microscope by a pathologist to check for signs of cancer.

Radiation therapy to the breast or chest to treat a previous cancer increases the risk of breast cancer.

Anything that increases a person’s chance of getting a disease is called a risk factor. Not every child with one or more risk factors will develop childhood breast cancer, and it will develop in some children who don’t have any known risk factors. Talk with your child’s doctor if you think your child may be at risk for breast cancer.

Risk factors for breast cancer in children, adolescents, and young adults include the following:

Genetic counseling may be done for patients with breast cancer and a family history of breast cancer.

It is not always clear from the family medical history whether a condition is inherited. Certain families may benefit from genetic counseling and genetic testing. Genetic counselors and other specially trained health professionals can help parents understand the following:

  • Their family medical history.
  • Their options for genetic testing for the BRCA1, BRCA2, or other genes that increase the risk for breast cancer.
  • The risk of cancer for the child and the child’s siblings.
  • The risks and benefits of learning genetic information.

Genetic counselors can also help parents cope with their child’s genetic testing results, including how to discuss the results with family members.

Signs of breast cancer include a lump or thickening in or near the breast.

These and other signs and symptoms may be caused by breast cancer or by other conditions.

Check with your child’s doctor if your child has any of the following:

  • A lump or thickening in or near the breast or in the underarm area.
  • A change in the size or shape of the breast.
  • A dimple or puckering in the skin of the breast.
  • A nipple turned inward into the breast.
  • Fluid, other than breast milk, from the nipples, including blood.
  • Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin that is around the nipple).
  • Dimples in the breast that look like the skin of an orange, called peau d’orange.

Tests that examine the breast are used to diagnose breast cancer.

In addition to asking about your child’s personal and family health history and doing a physical exam, your child’s doctor may perform the following tests and procedures.

  • Clinical breast exam (CBE): An exam of the breast by a doctor or other health professional. The doctor will carefully feel the breast and under the arm for lumps or anything else that seems unusual.
  • Mammogram: An x-ray of the breast. When treatment for another cancer included radiation therapy to the breast or chest, it is important to have a mammogram and MRI of the breast to check for breast cancer. These should be done beginning at age 25, or 8 years after finishing radiation therapy, whichever is later.
  • Breast MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of both breasts. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • 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.
  • 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.
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.

Stages of Childhood Breast Cancer

Key Points

  • There is no standard staging system for childhood breast cancer.
  • There are three ways cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.

There is no standard staging system for childhood breast cancer.

The process used to find out if cancer has spread from the breast to nearby areas or to other parts of the body is called staging. There is no standard staging system for childhood breast cancer. The results of tests and procedures done to diagnose breast cancer are used to help make decisions about treatment.

Sometimes childhood breast cancer recurs (comes back) in the breast or other parts of the body after treatment.

There are three ways cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • 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 breast cancer spreads to the bone, the cancer cells in the bone are actually breast cancer cells. The disease is metastatic breast cancer, not bone 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.

Treatment Option Overview

Key Points

  • There are different types of treatment for children and adolescents with breast tumors or cancer.
  • Children and adolescents with breast cancer should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
  • Two types of standard treatment are used for benign breast tumors:
    • Watchful waiting
    • Surgery
  • Three types of standard treatment are used for breast cancer:
    • Surgery
    • Radiation therapy
    • Chemotherapy
  • New types of treatment are being tested in clinical trials.
  • Treatment for childhood breast cancer 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 and adolescents with breast tumors or cancer.

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 and adolescents with breast cancer 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:

Two types of standard treatment are used for benign breast tumors:

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Benign breast tumors may disappear without treatment.

Surgery

Surgery is done to remove the tumor, but not the whole breast.

Three types of standard treatment are used for breast cancer:

Surgery

Surgery is done to remove the cancer, but not the whole breast.

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.

Chemotherapy

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 drug enters the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for childhood breast cancer may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit 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, including fertility 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. 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 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).

Treatment of Benign Childhood Breast Tumors

For more information about the treatments listed below, see the Treatment Option Overview section.

Treatment of newly diagnosed benign breast tumors in children may include the following:

  • Watchful waiting. These tumors may disappear without treatment.
  • Surgery to remove the tumor. Surgery may also be used for tumors that recur (come back).

Treatment of Childhood Breast Cancer

For more information about the treatments listed below, see the Treatment Option Overview section.

Treatment of newly diagnosed breast cancer in children may include the following:

Sometimes childhood breast cancer may come back after treatment. If your child is diagnosed with recurrent breast cancer, your child’s doctor will work with you to plan treatment.

For more information on the treatment of adolescents and young adults with breast cancer, see Breast Cancer Treatment (Adult).

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.

To Learn More About Childhood Breast Cancer

About This PDQ Summary

About PDQ

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 breast 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.

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 Breast Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/breast/patient/child-breast-treatment-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Breast Cancer—Patient Version

Breast Cancer—Patient Version

Overview

Breast cancer is the second most common cancer in women after skin cancer. Mammograms can detect breast cancer early, possibly before it has spread. Explore the links on this page to learn more about breast cancer prevention, screening, treatment, statistics, research, clinical trials, and more.

Coping with Cancer

The information in this section is meant to help you cope with the many issues and concerns that occur when you have cancer.

Emotions and Cancer Adjusting to Cancer Support for Caregivers Survivorship Advanced Cancer Managing Cancer Care

Childhood NUT Carcinoma (PDQ®)–Patient Version

Childhood NUT Carcinoma (PDQ®)–Patient Version

What is childhood NUT carcinoma?

NUT carcinoma (formerly called midline tract carcinoma) is a very rare and fast-growing type of cancer that can occur in children and adults. It forms in the squamous cells of the respiratory tract or other places along the middle of the body. The respiratory tract is made up of the nose, throat, larynx, trachea, bronchi, and lungs. NUT carcinoma may also form in other places along the middle of the body, such as the thymus, mediastinum (the area between the lungs), pancreas, liver, and bladder. It can spread to the lymph nodes, the lining around the lungs, bone marrow, or bone.

EnlargeDrawing showing areas where NUT carcinoma may form, including the nose (nasal cavity), throat (pharynx), larynx, lung, thymus, trachea, bronchi, area between the lungs, liver, pancreas, and bladder.
NUT carcinoma is a rare type of cancer that forms in the respiratory tract or in other places along the middle of the body, including the thymus, the area between the lungs, and the liver, pancreas, and bladder. It usually grows and spreads quickly and cannot be cured. NUT carcinoma usually occurs in children and young adults.

Causes and risk factors for childhood NUT carcinoma

Childhood NUT carcinoma is caused by a random change in genes, where part of the NUTM1 gene fuses with a part of another gene, usually BRD4, BRD3, or NSD3. The exact cause of these gene changes is unknown.

There are no known risk factors for developing this rare cancer. It is not known to run in families.

Symptoms of childhood NUT carcinoma

The symptoms of NUT carcinoma are not the same in every child. There may be no symptoms in the early stages. Symptoms such as fatigue and weight loss may appear as the tumor grows. Depending on where the tumor forms in the body, other symptoms—such as a lump, pain, cough, or shortness of breath—may occur.

These symptoms may be caused by problems other than childhood NUT carcinoma. The only way to know is to see your child’s doctor.

Tests to diagnose childhood NUT carcinoma

If your child has symptoms that suggest a NUT carcinoma, 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, the doctor may recommend other tests. If your child is diagnosed with NUT carcinoma, the results of these tests will help you and your child’s doctor plan treatment.

The tests used to diagnose childhood NUT carcinoma may include:

Chest x-ray

An x-ray is a type of radiation that can go through the body and onto film, making a picture of areas inside the body. A chest x-ray is one that makes pictures of the organs and bones inside the chest.

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 head and neck. This procedure is also called nuclear magnetic resonance imaging (NMRI).

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 taken from different angles. 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 of the head and neck; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.
Computed tomography (CT) scan of the head and neck. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.

Biopsy

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.

The following tests may be done on the sample of cells that was removed:

  • Immunohistochemistry is 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.
  • Cytogenetic analysis is a laboratory test in which the chromosomes of cells in a sample of bone marrow, blood, tumor, or other 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. Other tests, such as fluorescence in situ hybridization (FISH), may also be done to look for certain changes in the chromosomes.
  • Molecular testing checks for certain genes, proteins, or other molecules in a sample of tissue, blood, or other body fluid. A molecular test may be done with other procedures, such as biopsies, to help diagnose some types of cancer. 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 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.

Who treats children with NUT carcinoma?

A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for cancer. The pediatric oncologist works with other health care providers who are experts in treating children with cancer and also specialize in other areas of medicine. Other specialists may include:

Treatment of childhood NUT carcinoma

Because childhood NUT carcinoma is so rare, there is no standard treatment. Often, children receive a combination of treatments to try to control the growth and spread of the cancer:

  • When possible, surgery is done to remove as much of the tumor as possible.
  • Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Childhood NUT carcinoma is treated with external radiation therapy. This way of giving radiation uses a machine outside the body to send radiation toward the area of the body with cancer. Learn more about External Beam Radiation Therapy for Cancer and Radiation Therapy Side Effects.
  • Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cells or stops them from dividing. Chemotherapy drugs such as cisplatin, taxanes, and alkylating agents may be used. Learn more about Chemotherapy to Treat Cancer.

Even if the cancer responds to treatment, it will eventually continue to grow and spread. Your child’s doctor will talk with you about what to expect and possible next steps. If there are no treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible. Joining a clinical trial may be an option. Learn more about clinical trials in the next section.

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.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

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).

Prognosis of childhood NUT carcinoma

If your child has been diagnosed with NUT carcinoma, 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. Childhood NUT carcinoma grows and spreads quickly and is hard to treat. It is best to talk with your child’s cancer care team about your child’s prognosis.

Coping with your child's cancer

When your 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

About This PDQ Summary

About PDQ

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 NUT carcinoma. 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 NUT Carcinoma. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/midline/patient-child-midline-tract-carcinoma-treatment-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Childhood Laryngeal Tumors (PDQ®)–Patient Version

Childhood Laryngeal Tumors (PDQ®)–Patient Version

What are childhood laryngeal tumors?

Childhood laryngeal tumors form in the voice box, also called the larynx. These tumors may be benign (which means they are not cancer) or cancer. Most laryngeal tumors in children are not cancer and do not spread to other tissues. Both types of tumors need treatment.

The most common type of benign laryngeal tumor is papillomatosis of the larynx. In this condition, papillomas (benign tumors that look like warts) form in the lining of the larynx. These tumors may block the airway and cause trouble breathing. They often come back after treatment and, in rare cases, may turn into cancer in the larynx or the lung.

The larynx is a part of the throat, between the base of the tongue and the trachea (windpipe). The larynx contains the vocal cords, which vibrate and make sound when air is directed against them. The sound echoes through the pharynx, mouth, and nose to make a person’s voice.

There are three main parts of the larynx:

  • The supraglottis is the upper part of the larynx above the vocal cords, including the epiglottis.
  • The glottis is the middle part of the larynx where the vocal cords are located.
  • The subglottis is the lower part of the larynx between the vocal cords and the trachea.
EnlargeAnatomy of the larynx; drawing shows the epiglottis, supraglottis, vocal cord, glottis, and subglottis. Also shown are the tongue, trachea, and esophagus.
Anatomy of the larynx. The larynx is a part of the throat, between the base of the tongue and the trachea. The three main parts of the larynx are the supraglottis (including the epiglottis), the glottis (including the vocal cords), and the subglottis.

Causes of childhood laryngeal tumors

Laryngeal cancer in children is caused by certain changes to the way cells in the larynx 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?

Laryngeal papillomatosis is caused by infection with low-risk HPV (human papillomavirus), most often types 6 and 11. Children can get the HPV infection that causes laryngeal papillomatosis from an infected mother during birth. Most children with an HPV infection do not develop laryngeal papillomatosis. Talk with your child’s doctor if you think your child may be at risk.

Getting the HPV vaccine can help protect against HPV infection and reduce the risk of transmitting it during childbirth. Learn more about HPV vaccines.

Symptoms of childhood laryngeal tumors

Children may not have symptoms of a laryngeal tumor until the tumor has grown bigger. It’s important to check with your child’s doctor if your child has:

  • hoarseness or a change in the voice
  • trouble or pain when swallowing
  • trouble breathing
  • a high-pitched sound with breathing
  • a lump in the neck or throat
  • a sore throat
  • a cough that does not go away

Infants and young children with these tumors may grow slowly and not eat well or meet developmental milestones such as sitting, walking, and talking in sentences.

These symptoms may be caused by problems other than a laryngeal tumor. The only way to know is to see your child’s doctor.

Tests to diagnose childhood laryngeal tumors

If your child has symptoms that suggest a laryngeal tumor, the doctor will need to find out if these are due to cancer or some other 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 a laryngeal tumor, the results of these tests will help you and your child’s doctor plan treatment.

The tests used to diagnose laryngeal tumors may include:

Oral exam

In an oral exam, a doctor or dentist checks the mouth for abnormal areas. This exam may be done while under anesthesia. The doctor or dentist will feel the entire inside of the mouth with a gloved finger and examine the area with a small long-handled mirror and lights or a fiberoptic device.

Neck and chest x-ray

An x-ray is a type of radiation that can go through the body and make pictures of the inside of the body. A neck and chest x-ray makes pictures of the organs and bones inside the neck and chest.

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 head and neck. This procedure is also called nuclear magnetic resonance imaging (NMRI).

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. 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 tissues or organs 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 of the head and neck; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.
Computed tomography (CT) scan of the head and neck. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.

PET scan (positron emission tomography scan)

A PET scan uses a small amount of radioactive sugar (also called radioactive glucose) that is injected into the vein. Then a scanner is used to make detailed pictures of areas inside the body where the glucose is taken up. Because cancer calls often take up more glucose than normal cells, the pictures can be used to find cancer cells in the body.

EnlargePositron emission tomography (PET) scan; drawing shows a child lying on table that slides through the PET scanner.
Positron 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.

Ultrasound

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.

Barium swallow

Barium swallow is a series of x-rays of the esophagus and stomach. For this procedure, the patient drinks a liquid that contains barium (a silver-white metallic compound). The barium coats the esophagus and stomach which helps them show up more clearly in x-rays. This procedure is also called an upper GI series.

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 disease, such as cancer or laryngeal papillomatosis.

The sample of tissue may be removed during a procedure such as:

  • Laryngoscopy is a procedure in which the doctor checks the larynx (voice box) with a mirror or a laryngoscope to check for abnormal areas. A laryngoscope is a thin, tube-like instrument with a light and a lens for viewing the inside of the throat and voice box. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of disease.
  • Endoscopy is a procedure to look at organs and tissues inside the body, such as the throat, esophagus, and trachea to check for abnormal areas. An endoscope is a thin, lighted tube with a light and a lens for viewing that is inserted through an opening in the body, such as the mouth. A special tool on the endoscope may be used to remove samples of tissue.

Human papillomavirus (HPV) test

An HPV test checks tissue samples from the biopsy for certain types of HPV infection that may be linked to laryngeal papillomatosis.

Immunohistochemistry

An immunohistochemistry test measures 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.

Getting a second opinion

You may want to get a second opinion to confirm your child’s 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. 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.

Treatment of childhood laryngeal tumors

Who treats children with laryngeal tumors?

A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of laryngeal tumors. 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 with laryngeal tumors. You and your child’s care team will work together to decide on treatment. Many factors will be considered, such as your child’s overall health and whether the tumor is newly diagnosed or has come back.

Your child’s treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s 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 of childhood laryngeal cancer

Laser surgery uses a laser beam (a narrow beam of intense light) to destroy the cancer cells.

For laryngeal cancer that is likely to spread, external radiation therapy is also used. This treatment uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Learn more about External Beam Radiation Therapy for Cancer and Radiation Therapy Side Effects.

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 treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.

Treatment of childhood laryngeal papillomatosis

Laser surgery, which uses a laser beam (a narrow beam of intense light) to destroy cancer cells, may be used to treat newly diagnosed papillomatosis or other benign tumors.

For laryngeal papillomatosis that comes back after being removed by laser surgery four times in one year, treatment may include:

  • Immunotherapy. Immunotherapy uses the patient’s immune system to fight disease.
  • Laser surgery combined with 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.

Clinical trials

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 existing 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 that are 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.

Learn more at Clinical Trials Information for Patients and Caregivers.

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 these 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 come back.

Coping with your child's cancer

When your 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

About This PDQ Summary

About PDQ

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 laryngeal cancer and papillomatosis. 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 Laryngeal Tumors. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/head-and-neck/patient/child/laryngeal-treatment-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Biomarker Testing for Cancer Treatment

Biomarker Testing for Cancer Treatment

Doctor and patient looking at DNA genomic sequence on iPad

Biomarker testing (also called tumor testing, tumor profiling, or tumor genetic testing) finds changes in your cancer that could help you and your doctor choose your cancer treatment.

Credit: Darryl Leja, NHGRI

What is biomarker testing for cancer treatment?

Biomarker testing is a way to look for genes, proteins, and other substances (called biomarkers or tumor markers) that can provide information about cancer. Each person’s cancer has a unique pattern of biomarkers. Some biomarkers affect how certain cancer treatments work. Biomarker testing may help you and your doctor choose a cancer treatment for you.

There are also other kinds of biomarkers that can help doctors diagnose and monitor cancer during and after treatment. To learn more, visit the Tumor Markers fact sheet.

Biomarker testing is for people who have cancer. People with solid tumors and people with blood cancer can get biomarker testing.

Biomarker testing for cancer treatment may also be called:

  • tumor testing 
  • tumor genetic testing 
  • genomic testing or genomic profiling
  • molecular testing or molecular profiling 
  • somatic testing 
  • tumor subtyping 

A biomarker test may be called a companion diagnostic test if it is paired with a specific treatment.

Biomarker testing is different from genetic testing that is used to find out if someone has inherited mutations that make them more likely to get cancer. Inherited mutations are those you are born with. They are passed on to you by your parents.

How are biomarker tests used to select cancer treatment?

Biomarker tests can help you and your doctor select a cancer treatment for you. Some cancer treatments, including targeted therapies and immunotherapies, may only work for people whose cancers have certain biomarkers.

For example, people with cancer that has certain genetic changes in the EGFR gene can get treatments that target those changes, called EGFR inhibitors. In this case, biomarker testing can find out whether someone’s cancer has an EGFR gene change that can be treated with an EGFR inhibitor.

Biomarker testing could also help you find a study of a new cancer treatment (a clinical trial) that you may be able to join. Some studies enroll people based on the biomarkers in their cancer, instead of where in the body the cancer started growing. These are sometimes called basket trials. 

For some other clinical trials, biomarker testing is part of the study.  For example, studies like NCI-MATCH and NCI-COG Pediatric MATCH are using biomarker tests to match people to treatments based on the genetic changes in their cancers.

To find out if there are open trials for which you may be eligible, use the search tool at Find Clinical Trial. Or, contact the Cancer Information Service for help.

Is biomarker testing part of precision medicine?

Yes, biomarker testing is an important part of precision medicine, also called personalized medicine. Precision medicine is an approach to medical care in which disease prevention, diagnosis, and treatment are tailored to the genes, proteins, and other substances in your body.

For cancer treatment, precision medicine means using biomarker and other tests to select treatments that are most likely to help you, while at the same time sparing you from getting treatments that are not likely to help.

The idea of precision medicine isn’t new, but recent advances in science and technology have helped speed up the pace of this area of research. Scientists now understand that cancer cells can have many different changes in genes, proteins, and other substances that make the cells grow and spread. They have also learned that even two people with the same type of cancer may not have the same changes in their cancer. Some of these changes affect how certain cancer treatments work. 

Even though researchers are making progress every day, the precision medicine approach to cancer treatment is not yet part of routine care for most patients. But it’s important to note that even the “standard” approach to cancer treatment (selecting treatments based on the type of cancer you have, its size, and whether it has spread) is effective and is personalized to each patient. 

Should I get biomarker testing to select cancer treatment?

Talk with your health care provider to discuss whether biomarker testing for cancer treatment should be part of your care. Doctors usually suggest genomic biomarker testing (also called genomic profiling) for people with cancer that has spread or come back after treatment (what’s called advanced cancer). 

Biomarker testing is also done routinely to select treatment for people who are diagnosed with certain types of cancer—including non-small cell lung cancer, breast cancer, and colorectal cancer. 

It’s also a good idea to check with your health insurance provider to see if they will cover biomarker testing for your cancer. Biomarker testing is not available at every hospital. Check with your health care provider to see if biomarker testing is offered at the hospital or place where you get your cancer care.

How is biomarker testing done?

If you and your health care providers decide to make biomarker testing part of your care, they will take a sample of your cancer cells. If you have a solid tumor, they may take a sample during surgery. If you aren’t having surgery, you may need to have a biopsy of your tumor. 

If you have blood cancer or are getting a biomarker test known as a liquid biopsy, you will need to have a blood draw. You might get a liquid biopsy test if you can’t safely get a tumor biopsy, for example, because your tumor is hard to reach with a needle. 

Your samples will be sent to a special lab where they will be tested for certain biomarkers. The lab will create a report that lists the biomarkers in your cancer cells and if there are any treatments that might work for you. Your health care team will discuss the results with you to decide on a treatment. 

For some biomarker tests that analyze genes, you will also need to give a sample of your healthy cells. This is usually done by collecting your blood, saliva, or a small piece of your skin. These tests compare your cancer cells with your healthy cells to find genetic changes (called somatic mutations) that arose during your lifetime. Somatic mutations cause most cancers and can’t be passed on to family members.

Are there different types of biomarker tests?

Yes, there are many types of biomarker tests that can help select cancer treatment. Most biomarker tests used to select cancer treatment look for genetic markers. But some look for proteins or other kinds of markers.

Some tests check for a single biomarker. Others check for many biomarkers at the same time and may be called multigene tests or panel tests. One example is the Oncotype DX test, which looks at the activity of 21 different genes to predict whether chemotherapy is likely to work for someone with breast cancer. 

Some tests are for people with a certain type of cancer, like melanoma. Other tests look for biomarkers that are found in many cancer types, and such tests can be used by people with different kinds of cancer. 

Some tests, called whole-exome sequencing, look at all the genes in your cancer. Others, called whole-genome sequencing, look at all the DNA (both genes and outside of genes) in your cancer. 

Still other biomarker tests look at the number of genetic changes in your cancer (what’s known as tumor mutational burden). This information can help figure out if a type of immunotherapy known as immune checkpoint inhibitors may work for you.

Biomarker tests known as liquid biopsies look in blood or other fluids for biomarkers from cancer cells. There are two liquid biopsy tests approved by the Food and Drug Administration (FDA), called Guardant360 CDx and FoundationOne Liquid CDx

What do the results of a biomarker test mean?

The results of a biomarker test could show that your cancer has a certain biomarker that is targeted by a known therapy. That means that the therapy may work to treat your cancer. The matching therapy may be available as an FDA-approved treatment, an off-label treatment, or through participation in a clinical trial. 

The results could also show that your cancer has a biomarker that may prevent a certain therapy from working. This information could spare you from getting a treatment that won’t help you.

In many cases, biomarker testing may find changes in your cancer that won’t help your doctor make treatment decisions. For example, genetic changes that are thought to be harmless (benign) or whose effects are not known (variant of unknown significance) are not used to make treatment decisions. 

Based on your test results, your health care provider may recommend a treatment that is not FDA approved for your cancer type, but is approved for the treatment of a different type of cancer that has the same biomarker as your cancer. This means the treatment would be used off label, but it may work for you because your cancer has the biomarker that the treatment targets.

Some biomarker tests can find genetic changes that you may have been born with (inherited) that increase your risk of cancer or other diseases. These genetic changes are also called germline mutations. If such a change is found, you may need to get another genetic test to confirm whether you truly have an inherited mutation that increases cancer risk

Finding out that you have an inherited mutation that increases cancer risk may affect you and your family. For that reason, your health care provider may recommend that you speak with a genetic healthcare provider (such as a genetic counselor, clinical geneticist, or a certified genetic nurse) to help you understand what the test results mean for you and your family.

Will biomarker testing for cancer treatment help me?

Biomarker tests don’t help everyone who gets them. There are several different reasons why they may not help you. Biomarker testing may not help you if:

  • you are unable to safely get a biopsy needed for testing.
  • there is not enough tumor tissue in your biopsy sample to have biomarker testing done.
  • the test doesn’t find any biomarkers in your cancer that match with available therapies.
  • the test identifies a matching therapy that would be used off label, and your insurance doesn’t cover the cost.
  • the test identifies a matching therapy that is being tested in a clinical trial, and you are not able to participate in the trial.

Even if your test finds a biomarker that matches an available treatment, the therapy may not work for you. Sometimes other features of your cancer or your body affect how well a treatment works, such as how the medicine is broken down in your body. 

Another reason the treatment might not work is that not all of your cancer cells have the same biomarkers. That means that a biomarker test may find a treatment that will kill some, but not all, of your cancer cells. Cancer cells that are not killed by the treatment could keep growing, preventing the treatment from working or causing the cancer to quickly come back.

One other reason biomarker tests might not help is because the biomarkers in your cancer can change over time. But a test only captures a “snapshot” of the changes at one point in time. So, the results of a biomarker test done in the past may not reflect the biomarkers in your cancer now. Your health care provider may want to test your cancer again, for example, if it comes back after treatment. 

How much does biomarker testing for cancer treatment cost?

The cost of biomarker testing varies widely depending on the type of test you get, the type of cancer you have, and your insurance plan. 

For people with advanced cancer, some biomarker tests are covered by Medicare and Medicaid. Private insurance providers often cover the cost of a biomarker test if there is enough proof that the test is required to guide treatment decisions. Tests without enough proof to support their value may be considered experimental and are likely not covered by insurance.

Many clinical trials involve biomarker testing. If you join one of these clinical trials, the cost of biomarker testing might be covered. The study coordinator can give you more information about related costs.

Radiation Therapy to Treat Cancer

Radiation Therapy to Treat Cancer

Technician positions a person lying on a treatment table for radiation therapy.

Radiation therapy kills cancer cells or slows their growth by damaging their DNA.

Credit: National Cancer Institute

Radiation therapy (also called radiotherapy) is a cancer treatment that uses high doses of radiation to kill cancer cells and shrink tumors. At low doses, radiation is used in x-rays to see inside your body, as with x-rays of your teeth or broken bones.

How radiation therapy works against cancer

At high doses, radiation therapy kills cancer cells or slows their growth by damaging their DNA. Cancer cells whose DNA is damaged beyond repair stop dividing or die. When the damaged cells die, they are broken down and removed by the body.

Radiation therapy does not kill cancer cells right away. It takes days or weeks of treatment before DNA is damaged enough for cancer cells to die. Then, cancer cells keep dying for weeks or months after radiation therapy ends.

Types of radiation therapy

There are two main types of radiation therapy, external beam and internal.

The type of radiation therapy that you may have depends on many factors, including:

  • the type of cancer
  • the size of the tumor
  • the tumor’s location in the body
  • how close the tumor is to normal tissues that are sensitive to radiation
  • your general health and medical history
  • whether you will have other types of cancer treatment
  • other factors, such as your age and other medical conditions

External beam radiation therapy

External beam radiation therapy comes from a machine that aims radiation at your cancer. The machine is large and may be noisy. It does not touch you, but can move around you, sending radiation to a part of your body from many directions.

External beam radiation therapy is a local treatment, which means it treats a specific part of your body. For example, if you have cancer in your lung, you will have radiation only to your chest, not to your whole body.

Learn more about external beam radiation therapy.

Internal radiation therapy

Internal radiation therapy is a treatment in which a source of radiation is put inside your body. The radiation source can be solid or liquid.

Internal radiation therapy with a solid source is called brachytherapy. In this type of treatment, seeds, ribbons, or capsules that contain a radiation source are placed in your body, in or near the tumor. Like external beam radiation therapy, brachytherapy is a local treatment and treats only a specific part of your body.

With brachytherapy, the radiation source in your body will give off radiation for a while.

Learn more about brachytherapy.

Internal radiation therapy with a liquid source is called systemic therapy. Systemic means that the treatment travels in the blood to tissues throughout your body, seeking out and killing cancer cells. You receive systemic radiation therapy by swallowing, through a vein via an IV line, or through an injection.

With systemic radiation, your body fluids, such as urine, sweat, and saliva, will give off radiation for a while.

Why people with cancer receive radiation therapy

Radiation therapy is used to treat cancer and ease cancer symptoms.

When used to treat cancer, radiation therapy can cure cancer, prevent it from returning, or stop or slow its growth.

When treatments are used to ease symptoms, they are known as palliative treatments. External beam radiation may shrink tumors to treat pain and other problems caused by the tumor, such as trouble breathing or loss of bowel and bladder control.

Pain from cancer that has spread to the bone can be treated with systemic radiation therapy drugs called radiopharmaceuticals.

Types of cancer that are treated with radiation therapy

External beam radiation therapy is used to treat many types of cancer.

Brachytherapy is most often used to treat cancers of the head and neck, breast, cervix, prostate, and eye.

A systemic radiation therapy called radioactive iodine, or I-131, is most often used to treat certain types of thyroid cancer.

Another type of systemic radiation therapy, called targeted radionuclide therapy, is used to treat some patients who have advanced prostate cancer or gastroenteropancreatic neuroendocrine tumor (GEP-NET). This type of treatment may also be referred to as molecular radiotherapy.

How radiation is used with other cancer treatments

For some people, radiation may be the only treatment you need. But, most often, you will have radiation therapy with other cancer treatments, such as surgerychemotherapy, and immunotherapy. Radiation therapy may be given before, during, or after these other treatments to improve the chances that treatment will work. The timing of when radiation therapy is given depends on the type of cancer being treated and whether the goal of radiation therapy is to treat the cancer or ease symptoms.

When radiation is combined with surgery, it can be given:

  • Before surgery, to shrink the size of the cancer so it can be removed by surgery and be less likely to return.
  • During surgery, so that it goes straight to the cancer without passing through the skin. Radiation therapy used this way is called intraoperative radiation. With this technique, doctors can more easily protect nearby normal tissues from radiation.
  • After surgery to kill any cancer cells that remain.

Lifetime dose limits

There is a limit to the amount of radiation an area of your body can safely receive over the course of your lifetime. Depending on how much radiation an area has already been treated with, you may not be able to have radiation therapy to that area a second time. But, if one area of the body has already received the safe lifetime dose of radiation, another area might still be treated if the distance between the two areas is large enough.

Radiation therapy can cause side effects

Radiation not only kills or slows the growth of cancer cells, it can also affect nearby healthy cells. Damage to healthy cells can cause side effects.

Learn more about the side effects of radiation therapy.

How much radiation therapy costs

Radiation therapy can be expensive. It uses complex machines and involves the services of many health care providers. The exact cost of your radiation therapy depends on the cost of health care where you live, what type of radiation therapy you get, and how many treatments you need.

Talk with your health insurance company about what services it will pay for. Most insurance plans pay for radiation therapy. To learn more, talk with the business office at the clinic or hospital where you go for treatment. If you need financial assistance, there are organizations that may be able to help. To find such organizations, go to the National Cancer Institute database, Organizations that Offer Support Services and search for “financial assistance.” Or call toll-free 1-800-4-CANCER (1-800-422-6237) to ask for information on organizations that may help.

Special diet needs while on radiation therapy

Radiation can cause side effects that make it hard to eat, such as nausea, mouth sores, and throat problems called esophagitis. Since your body uses a lot of energy to heal during radiation therapy, it is important that you eat enough calories and protein to maintain your weight during treatment.

If you are having trouble eating and maintaining your weight, talk to your doctor or nurse. You might also find it helpful to speak with a dietitian. For more information about coping with eating problems see the booklet Eating Hints or read more about side effects.

Working during radiation therapy

Some people are able to work full-time during radiation therapy. Others can work only part-time or not at all. How much you are able to work depends on how you feel. Ask your doctor or nurse what you may expect from the treatment you will have.

You are likely to feel well enough to work when you first start your radiation treatments. As time goes on, do not be surprised if you are more tired, have less energy, or feel weak. Once you have finished treatment, it may take just a few weeks for you to feel better—or it could take months.

You may get to a point during your radiation therapy when you feel too sick to work. Talk with your employer to find out if you can go on medical leave. Check that your health insurance will pay for treatment while you are on medical leave.

Childhood Salivary Gland Tumors (PDQ®)–Patient Version

Childhood Salivary Gland Tumors (PDQ®)–Patient Version

What are salivary gland tumors?

Salivary gland tumors are abnormal growths that can form in the salivary glands. They can be benign (not cancer) or malignant (cancerous). Although benign tumors do not spread to other parts of the body, they may require treatment to stop them from continuing to grow and press on nearby tissue. Cancerous tumors can spread to other areas of the body and will be treated to kill the cancer cells. Salivary gland tumors rarely occur in children.

The salivary glands make saliva and release it into the mouth. Saliva has enzymes that help digest food and antibodies that help protect against mouth and throat infections. There are three pairs of major salivary glands:

  • Parotid glands are the largest glands and are found in front of and just below each ear. Most salivary gland tumors begin in this gland.
  • Sublingual glands are found under the tongue in the floor of the mouth.
  • Submandibular glands are found below the jawbone.
EnlargeAnatomy of the salivary glands; drawing shows a cross section of the head and the three main pairs of salivary glands. The parotid glands are in front of and just below each ear; the sublingual glands are under the tongue in the floor of the mouth; and the submandibular glands are below each side of the jawbone. The tongue and lymph nodes are also shown.
Anatomy of the salivary glands. The three main pairs of salivary glands are the parotid glands, the sublingual glands, and the submandibular glands.

Causes and risk factors for childhood salivary gland tumors

Salivary gland tumors in children are caused by certain changes to the way salivary gland 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. Past treatment for cancer with chemotherapy or radiation therapy is a risk factor for childhood salivary gland tumors. Not every child with this risk factor will develop a salivary gland tumor. And it will develop in some children who don’t have a known risk factor. Talk with your child’s doctor if you think your child may be at risk.

Symptoms of childhood salivary gland tumors

Children may not have symptoms of a salivary gland tumor until the tumor has grown bigger. It’s important to check with your child’s doctor if your child has:

  • a lump near the ear, cheek, jaw, lip, or inside the mouth that may be painless
  • numbness or weakness in the face
  • pain in the face that does not go away

These symptoms may be caused by problems other than a salivary gland tumor. The only way to know is to see your child’s doctor.

Tests to diagnose childhood salivary gland tumors

If your child has symptoms that suggest a salivary gland tumor, the doctor will need to find out if these are due to a salivary gland tumor 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 a salivary gland tumor, the results of these tests will help you and your child’s doctor plan treatment.

The tests used to diagnose salivary gland tumors in children may include:

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 head and neck. This procedure is also called nuclear magnetic resonance imaging (NMRI).

EnlargeMagnetic resonance imaging (MRI) scan; drawing shows a child lying on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body.
Magnetic 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.

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. 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 of the head and neck; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.
Computed tomography (CT) scan of the head and neck. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.

Ultrasound exam

An ultrasound exam uses high-energy sound waves (ultrasound) which bounce off internal tissue or organs, such as the pelvis, and make echoes. The echoes form a picture of body tissues called a sonogram.

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. Fine-needle aspiration biopsy is used to check for salivary gland cancer. During a fine-needle aspiration biopsy, tissue or fluid is removed using a thin needle.

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 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, 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 salivary gland tumors?

A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of salivary gland tumors. 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:

Treatment of childhood salivary gland tumors

There are different types of treatment for children and adolescents with salivary gland tumors. You and your child’s care team will work together to decide treatment. Many factors will be considered, such as your child’s overall health and whether the tumor is newly diagnosed or has come back.

Your child’s treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child’s 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 of salivary gland tumors may include:

  • Surgery to remove salivary gland tumors or salivary gland cancer is the most common treatment. If the parotid gland is removed during surgery, special care must be taken to avoid damage to the facial nerve. Learn more about Surgery to Treat Cancer.
  • Radiation therapy may be given after surgery for salivary gland cancer. Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Salivary gland cancer may be treated with external beam radiation therapy or internal radiation therapy if the cancer is likely to spread.
    • External beam radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
    • Internal radiation therapy, also called brachytherapy, uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

    Learn more about Radiation Therapy to Treat Cancer and Radiation Therapy Side Effects.

  • Targeted therapy (entrectinib or larotrectinib) may be used to treat recurrent childhood salivary gland cancer. Recurrent salivary gland cancer is cancer that has come back after it has been treated. 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. The doctor may suggest biomarker tests to help predict your child’s response to certain targeted therapy drugs. Learn more about Biomarker Testing for Cancer Treatment and Targeted Therapy to Treat Cancer.

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.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

Prognosis and prognostic factors for childhood salivary gland tumors

If your child has been diagnosed with salivary gland 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 has spread to lymph nodes or other parts of the body at the time of diagnosis and whether the cancer can be completely removed by surgery. The prognosis for salivary gland cancer in children is usually good.

No two people are alike, and responses to treatment can vary greatly. Your child’s cancer care team is in the best position to talk with you about your child’s prognosis.

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.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

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, such as:
    • dry mouth
    • trouble seeing
    • numbness or weakness in the face
    • changes in the way the bones of the head and face grow
    • other changes in the child’s appearance
  • changes in mood, feelings, thinking, learning, or memory
  • second cancers (new types of cancer)

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 tumor 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 tumor 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, see Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.

Related resources

About This PDQ Summary

About PDQ

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 salivary gland 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.

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 Salivary Gland Tumors. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/head-and-neck/patient/child/salivary-gland-treatment-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.

Childhood Oral Cavity Cancer (PDQ®)–Patient Version

Childhood Oral Cavity Cancer (PDQ®)–Patient Version

What is childhood oral cavity cancer?

Childhood oral cavity cancer is a rare type of cancer that forms in the mouth. There are several types of oral cavity cancers, including lymphoma, sarcoma, squamous cell carcinoma, and mucoepidermoid carcinoma. Most of the tumors (more than 90%) that children get in their mouth are not cancer. All types of tumors in the mouth can affect eating or speaking and need treatment.

The oral cavity includes:

  • the front two-thirds of the tongue
  • the gums (gingiva)
  • the lining of the inside of the cheeks (buccal mucosa)
  • the bottom (floor) of the mouth under the tongue
  • the roof of the mouth (hard palate)
  • the small area behind the wisdom teeth (retromolar trigone)
EnlargeAnatomy of the oral cavity; drawing shows the lip, hard palate, soft palate, retromolar trigone, front two-thirds of the tongue, gingiva, buccal mucosa, and floor of mouth. Also shown are the teeth, uvula, and tonsil.
Anatomy of the oral cavity. The oral cavity includes the lips, hard palate (the bony front portion of the roof of the mouth), soft palate (the muscular back portion of the roof of the mouth), retromolar trigone (the area behind the wisdom teeth), front two-thirds of the tongue, gingiva (gums), buccal mucosa (the inner lining of the lips and cheeks), and floor of the mouth under the tongue.

Causes and risk factors for childhood oral cavity cancer

Oral cavity cancer is caused by certain changes to the way the cells in the oral cavity 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 oral cavity cancer. And it will develop in some children who don’t have a known risk factor.

Risk factors for oral cavity cancer in children and adolescents include:

Getting the HPV vaccine can protect against HPV infection and lower the risk of this and many other types of cancer. Learn more about HPV and Cancer.

Talk with your child’s doctor if you think your child may be at risk.

Symptoms of childhood oral cavity cancer

The symptoms of oral cavity cancer may be similar to an infection. It’s important to check with your child’s doctor if your child has:

  • a sore in the mouth that does not heal
  • a lump or thickening in the mouth
  • a white or red patch on the gums, tongue, or lining of the mouth
  • bleeding or pain in the mouth

These symptoms may be caused by problems other than oral cavity cancer. The only way to know is to see your child’s doctor.

Tests to diagnose childhood oral cavity cancer

If your child has symptoms that suggest an oral cavity 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 health history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with oral cavity cancer, the results of these tests will help you and your child’s doctor plan treatment.

The tests used to diagnose oral cavity cancer may include:

Oral exam

In an oral exam, a medical doctor or dentist checks the mouth for abnormal areas. This exam may be done while under anesthesia. The doctor or dentist will feel the entire inside of the mouth with a gloved finger and examine the oral cavity with a small long-handled mirror and lights or a fiberoptic device. This will include checking the insides of the cheeks and lips; the gums; the roof and floor of the mouth; and the top, bottom, and sides of the tongue. The neck will be felt for swollen lymph nodes. A dental exam may also be done.

X-ray

An x-ray is a type of radiation that can go through the body and make 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 head and neck. This procedure is also called nuclear magnetic resonance imaging (NMRI).

EnlargeMagnetic resonance imaging (MRI) scan; drawing shows a child lying on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body.
Magnetic 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.

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. 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 tissues or organs show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. To learn more, see Computed Tomography (CT) Scans and Cancer.

EnlargeComputed tomography (CT) scan of the head and neck; drawing shows a child lying on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.
Computed tomography (CT) scan of the head and neck. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of the inside of the head and neck.

PET scan (positron emission tomography scan)

A PET scan uses a small amount of radioactive sugar (also called radioactive glucose) that is injected into the vein. Then a scanner is used to make detailed, computerized pictures of areas inside the body where the glucose is taken up. Because cancer cells often take up more glucose than normal cells, the pictures can be used to find cancer cells in the body.

EnlargePositron emission tomography (PET) scan; drawing shows a child lying on table that slides through the PET scanner.
Positron 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.

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.

  • Fine-needle aspiration biopsy is the removal of cells, tissue or fluid using a thin needle.
  • Incisional biopsy is the surgical removal of part of a lump or a sample of tissue that doesn’t look normal.

Getting a second opinion

You may want to get a second opinion to confirm your child’s 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. 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 oral cavity cancer?

A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of oral cavity 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:

Treatment of childhood oral cavity cancer

There are different types of treatment for children with oral cavity cancer. You and your child’s care team will work together to decide treatment. Many factors will be considered, such as where the cancer is located and your child’s age and overall health.

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 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.

For oral cavity cancer in children, treatment might include:

  • Surgery is the most common treatment for oral cavity cancer in children. For children with an oral cavity tumor that is not cancer, surgery is likely the only treatment that will be needed. Learn more about Surgery to Treat Cancer.
  • Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. Chemotherapy may be given with other types of treatments.

    Chemotherapy can be given in different ways. For oral cavity cancer, chemotherapy is injected into a vein, enabling it to reach cancer cells throughout the body. Learn more about Chemotherapy to Treat Cancer.

  • Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. The type of radiation therapy most often used for oral cavity cancer is external beam radiation therapy. This treatment uses a machine outside the body to send radiation toward the area of the body with cancer. Learn more about External Beam Radiation Therapy for Cancer and Radiation Therapy Side Effects.

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.

Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.

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 changes or if the cancer has come back.

Coping with your child's cancer

When your 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

About This PDQ Summary

About PDQ

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 oral cavity 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.

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 Oral Cavity Cancer. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: /types/head-and-neck/patient/child/oral-cavity-treatment-pdq. Accessed <MM/DD/YYYY>.

Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.

Disclaimer

The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.