Childhood Tracheobronchial Tumors Treatment (PDQ®): Treatment - Patient Information [NCI]

General Information About Childhood Tracheobronchial Tumors

Tracheobronchial tumor is a type of childhood lung cancer that forms in the lining of the trachea or bronchi.

Most tracheobronchial tumors begin in the inside lining of the trachea (windpipe) or large 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. They are usually benign (not cancer) in children and malignant (cancer) in adults.

There are several types of tracheobronchial tumors or cancers, such as the following:

• Carcinoid tumor (most common in children) can be benign or malignant.
• Mucoepidermoid carcinoma is a slow-growing tumor.
• Inflammatory myofibroblastic tumor is a slow-growing tumor.
• Rhabdomyosarcoma (malignant).
• Granular cell tumor is a benign tumor.

Most tracheobronchial tumors in children are malignant (cancer). Sometimes, a slow-growing tracheobronchial tumor, such as inflammatory myofibroblastic tumor, becomes cancer that may spread to other parts of the body.

Signs and symptoms of a tracheobronchial tumor include wheezing and dry cough.

Tracheobronchial tumors may cause any of the following signs and symptoms. Check with your child's doctor if your child has any of the following:

• 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.

Conditions other than tracheobronchial tumors may cause these same signs and symptoms. For example, symptoms of tracheobronchial tumors are a lot like the symptoms of asthma, which can make it hard to diagnose the tumor.

Tests that examine the trachea and bronchi are used to help diagnose tracheobronchial tumor.

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:

• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck and chest, 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.
  
  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.
• Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.

Rarely, the following tests and procedures may be used:

• Bronchography: 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 injected or put through a bronchoscope to coat the airways and make them show up more clearly on x-ray film.
• Octreotide scan: 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.

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

Treatment options and prognosis depend on the following:

• The type of tracheobronchial tumor.
• Whether the tumor 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 recurred (come back).

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 require more aggressive treatment.

Stages of Tracheobronchial Tumors

There is no standard staging system for childhood tracheobronchial tumors.

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

Sometimes childhood tracheobronchial tumors recur (come back) after treatment.

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 a tumor in the trachea or bronchi spreads to the liver, the cancer cells in the liver are actually from the trachea or bronchi. The disease is metastatic tracheobronchial cancer, not liver cancer.

Treatment Option Overview

There are different types of treatment for children with a tracheobronchial tumor.

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 a tracheobronchial tumor should have their treatment planned by a team of doctors who are experts in treating childhood diseases.

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:

• Pediatrician.
• Pediatric surgeon.
• Radiation oncologist.
• Pathologist.
• Pediatric nurse specialist.
• Social worker.
• Rehabilitation specialist.
• Psychologist.
• Child-life specialist.
• Fertility specialist.

Four types of standard treatment are used:

Surgery

Surgery to remove the tumor is used to treat all types of tracheobronchial tumor except rhabdomyosarcoma. Sometimes a type of surgery called a sleeve resection is used. The tumor and lymph nodes and vessels where cancer has spread are removed.

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 is used to treat rhabdomyosarcoma. For more information, see Drugs Approved for Rhabdomyosarcoma.

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.

Radiation therapy is used to treat rhabdomyosarcoma.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells.

• Tyrosine kinase inhibitors: These targeted therapy drugs block signals needed for tumors to grow. Crizotinib is used to treat inflammatory myofibroblastic tumors in the trachea or bronchi that have a certain change in the ALKgene.

Targeted therapy is also being studied for the treatment of relapsed or refractory tracheobronchial tumors.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for childhood tracheobronchial tumor may cause side effects.

For information about side effects that begin during treatment for cancer, see our Side Effects page.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

• Physical problems.
• Changes in mood, feelings, thinking, learning, or memory.
• 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.

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.

Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Childhood Tracheobronchial Tumors

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

The treatment of tracheobronchial tumors depends on the type of cell the cancer formed from. Treatment of newly diagnosed tracheobronchial tumors in children may include the following:

• Surgery to remove the tumor, for all types of tracheobronchial tumors except rhabdomyosarcoma.
• Chemotherapy and radiation therapy, for rhabdomyosarcoma that forms in the trachea or bronchi. For more information about rhabdomyosarcoma and its treatment, see Childhood Rhabdomyosarcoma Treatment.
• Targeted therapy (crizotinib), for inflammatory myofibroblastic tumors that form in the trachea or bronchi.

For information about the treatment of tracheobronchial carcinoid tumors, see Childhood Gastrointestinal Neuroendocrine Tumors Treatment.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Treatment of Recurrent Childhood Tracheobronchial Tumors

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

Treatment of recurrent tracheobronchial tumors in children may include the following:

• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

To Learn More About Tracheobronchial Tumors

For more information from the National Cancer Institute about tracheobronchial tumors, see the following:

• Lung Cancer Home Page
• Computed Tomography (CT) Scans and Cancer
• Immunotherapy to Treat Cancer
• Targeted Therapy to Treat Cancer

For more childhood cancer information and other general cancer resources, see the following:

• About Cancer
• Childhood Cancers
• CureSearch for Children's Cancer
• Late Effects of Treatment for Childhood Cancer
• Adolescents and Young Adults with Cancer
• Children with Cancer: A Guide for Parents
• Cancer in Children and Adolescents
• Staging
• Coping with Cancer
• Questions to Ask Your Doctor about Cancer
• For Survivors and Caregivers

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.

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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.

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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.

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Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).

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

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Last Revised: 2023-01-19

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