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

Tracheobronchial tumors begin in the inside lining of the trachea or bronchi. Most tracheobronchial tumors in children are benign and occur in the trachea (windpipe) or large bronchi (large airways of the lung). Sometimes, a slow-growing tracheobronchial tumor, such as inflammatory myofibroblastic tumor, becomes cancer that may spread to other parts of the body.

There are several types of tumors or cancers that can form in the trachea or bronchi. These may include the following:

• Carcinoid tumor (most common in children).
• Mucoepidermoid carcinoma.
• Inflammatory myofibroblastic tumor.
• Rhabdomyosarcoma.
• Granular cell tumor.

Signs and symptoms of a tracheobronchial tumor include headache and blocked or stuffy nose.

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.
• Spitting up blood from the airways or lung.
• Frequent infections in the lung, such as pneumonia.
• Feeling very tired.
• Loss of appetite or weight loss for no known reason.

Other conditions that are not 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.

Primary lung tumors are rare in children and histologically quite diverse A review of primary malignant epithelial lung tumors using the National Cancer Data Base found that the most common primary malignant pediatric lung neoplasms were carcinoid tumors (63%) followed by mucoepidermoid carcinoma of the lung (18%).

Tracheobronchial tumors are a heterogeneous group of primary endobronchial lesions. Although adenoma implies a benign process, all varieties of tracheobronchial tumors occasionally display malignant behavior. Most primary lung tumors are malignant. In a review of 383 primary pulmonary neoplasms in children, 76% were malignant and 24% were benign.

The following histological types have been identified

• Carcinoid tumor (neuroendocrine tumor of the bronchus). Carcinoid tumors account for 80% to 85% of all tracheobronchial tumors in childre It is the most common tracheobronchial tumor.
• Mucoepidermoid carcinoma. A slow-growing vascular polypoid mass of the airway that is the second most common (10%) pediatric tracheobronchial tumor.
• Inflammatory myofibroblastic tumors. These low-grade tumors account for 1% of pediatric tracheobronchial tumors, are commonly located in the upper trachea, and rarely metastasize. Intrabronchial (pulmonary) inflammatory myofibroblastic tumors are incredibly rare compared with other sites of disease.
• Rhabdomyosarcoma.
• Granular cell tumors. Malignant transformation has not been documented in pediatric patients.

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

The following tests and procedures may be used:

• Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, 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.

• 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.

If cancer has formed in the trachea or bronchi, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.

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 diagnose cancer 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.

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.
• Targeted therapy (crizotinib), for inflammatory myofibroblastic tumors that form in the trachea or bronchi.

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.

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.

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. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.

• 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 ALK gene.

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.

Treatment for childhood tracheobronchial tumor may cause side effects.

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

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

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order 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 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.
• How much damage has been done to the lung tissue.
• 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 cancer is very good, unless the child has rhabdomyosarcoma.