Tracheal Diseases: Tracheal & Bronchial Tumors

The trachea (windpipe) is the airway that leads from the larynx (voice box) to the bronchi, which are airways that lead to the lungs.

Tumors that arise in the trachea (called primary tracheal tumors) and bronchi are very rare. These tumors account for just 2 percent of all upper airway tumors, and only 0.2 percent of all respiratory tract cancers. The vast majority of tracheal and bronchial tumors in adults are malignant (cancerous), but a few are benign (noncancerous).

The two most common types of malignant tracheal and bronchial tumors are squamous cell and adenoid cystic carcinoma. Another type of tumor, carcinoid tumor, is more common in the bronchi. Following are brief descriptions of these tumor types.

• Squamous Cell Carcinoma

  The most common type of tracheal tumor is squamous cell carcinoma, which is a fast-growing cancer that usually arises in the lower portion of the trachea. This type of tumor often penetrates the mucosal lining of the airway as it grows, which can cause ulceration and bleeding in the trachea. Squamous cell carcinoma is more common in men than in women. Smoking is the main risk factor for this type of cancer.

• Adenoid Cystic Carcinoma

  These slow-growing tumors eventually close off the airway as they progress, but are less likely to penetrate the mucosal lining of the trachea. Adenoid cystic carcinomas are found in equal numbers among men and women between the ages of 40 and 60. Unlike squamous cell carcinoma, smoking is not a risk factor for this type of cancer.

• Carcinoid Tumors

  These tumors arise from neuroendocrine cells, which produce hormones such as serotonin. Although carcinoid tumors are more commonly found in the gastrointestinal system, a few occur in the bronchi.

Some tracheal and bronchial tumors develop when cancer in another part of the body metastasizes (spreads) to the trachea or bronchi. This is called metastatic disease.

Although malignant, or cancerous, tracheal tumors are more common in adults, they are often benign when found in children. Some of the more common benign tracheal tumors include:

• Papillomas

  These cauliflower-like tumors are thought to be caused by the human papilloma virus (HPV). Papillomatosis refers to multiple papilloma tumors. Papillomas are the most common benign tracheal tumor in children.

• Chondromas

  The most common benign tracheal tumor, these firm nodules arise from the cartilage comprising the rings of the trachea. Chondromas occur more frequently in the larynx.

• Hemangiomas

  These growths are an abnormal buildup of capillaries in the trachea.

Symptoms

Patients with tracheal and bronchial tumors may experience the following symptoms:

• coughing
• coughing up blood (hemoptysis), which also may be a sign of metastatic disease that has spread from another part of the body
• difficult or labored breathing (dyspnea)
• wheezing
• stridor, a high-pitched musical sound that occurs as the breath is drawn in, which is caused by a blockage in the throat or voice box (larynx)

Patients with more advanced disease may experience difficulty swallowing (dysphagia) and hoarseness, which usually indicates that the cancer has grown beyond the trachea.

Symptoms of benign tracheal and bronchial tumors are similar to those of malignant tumors, including coughing, hemoptysis, wheezing, and stridor.

Diagnosis

Tracheal and bronchial tumors can be difficult to identify. Early signs and symptoms may progress incrementally over time and are often mistaken for a variety of other respiratory disorders, such as asthma, bronchitis, and chronic obstructive pulmonary disease (COPD). In addition, symptoms of benign and malignant tumors in this area are very similar, making it difficult to diagnose the type of tumor based solely on a patient's symptoms. Additional tests are required to help assess and diagnose tracheal and bronchial tumors. Unfortunately, many patients are diagnosed when their tumor has spread to nearby lymph nodes or other areas of the body.

Physicians at Memorial Sloan-Kettering have developed a CT-scanning method that captures a three-dimensional view of the airway. This imaging technique aids in visualizing the location and extent of the tumor before more invasive diagnostic procedures are performed. This information is critical in determining whether a patient may be a candidate for surgery or other treatments.

Additional tests are used to help confirm the diagnosis and stabilize the airway:

• Laryngoscopy

  A flexible, narrow tube (endoscope) that has a camera on the tip is inserted through the mouth into the windpipe to examine the upper portion of the airway.

• Bronchoscopy

  An endoscope designed for the airways is inserted through the mouth into the windpipe to examine the trachea and bronchi. Either a rigid or flexible bronchoscope can be used to diagnose and deliver treatments to tracheal and bronchial tumors.

• Biopsy

  A small sample of tissue is removed with a bronchoscope for examination under a microscope.

Before and after treatment, the doctor often measures respiratory function with the following tests:

• Spirometry, which measures how much air is expelled from the lungs, to determine the location of certain types of blockages.

• Six-minute walk to test endurance and oxygen levels in the blood.

Treatment

The doctors who make up Memorial Sloan-Kettering's multidisciplinary Complex Airway Program are experienced in selecting the appropriate treatment for patients with tracheal and bronchial tumors. Treatment may include surgery or bronchoscopic treatments, alone or in combination with radiation therapy.

Other treatments, including radiation therapy, bronchoscopic treatments, and palliative surgery, may help restore breathing and slow tumor progression in patients who are not candidates for surgical removal of the tumor. Chemotherapy, usually in combination with radiation therapy, is another treatment option for patients with large squamous cell tracheal tumors.

Palliative Therapies

Patients who are not candidates for surgery may require palliative therapies to help restore breathing and slow tumor progression. The surgeon may use a number of techniques to help improve breathing. One technique involves placing a tube made of metal, silicone, or other material, known as a tracheobronchial airway stent, in the trachea to keep the airway open. In rare cases, patients with extensive tumors may require a surgery known as tracheostomy. In this procedure, the surgeon inserts a tube into the patient's trachea through a small opening in the neck prior to other treatments.

Surgery

Surgical removal of the tumor (known as resection) is the preferred treatment for both malignant and benign tumors that are confined to less than half of the trachea. In these procedures, the surgeon will remove the tumor and a small margin (extra amount) of healthy tissue surrounding it, rejoining the ends of the trachea together.

Surgical removal of tracheal tumors can be a complex procedure. The blood supply to the trachea is delicate and easily damaged. Damage to the blood supply makes it more difficult for the trachea to heal, increasing the complications of tracheal surgery. Our surgeons are specially trained in surgical techniques to preserve the blood supply and reduce the risk of these complications, improving the chance of a successful outcome.

Bronchoscopic Treatments

Patients who are not candidates for surgery may benefit from a number of treatments that can be delivered through a bronchoscope, a flexible tube attached to a tiny camera that is inserted through the mouth. This is often performed in conjunction with a rigid bronchoscope. In some cases, bronchoscopic treatments may reduce the size of the tumor so that it can be surgically removed. Here are some of the most commonly used bronchoscopic treatments:

• Laser Therapy

  Tumor tissue is vaporized with a highly focused beam of light to remove the tumor and open the airway.

• Spray Cryotherapy

  This treatment involves applying liquid nitrogen to the tumor, freezing it so that the surgeon can remove it. Spray cryotherapy also may be used to stop bleeding in patients who cough up blood, or during bronchoscopic removal of the tumor.

• Brachytherapy

  This type of radiation therapy uses a bronchoscope to help guide radiation directly to the tumor site, minimizing damage to nearby healthy tissue. Brachytherapy may be selected when the patient is not eligible for external-beam radiation therapy (described below).

• Photodynamic Therapy

  A strong light activates a photosensitive chemical that is applied to the tumor, which destroys abnormal tissue while causing minimal damage to surrounding tissue.

• Argon Beam Coagulation

  Similar to laser therapy, this treatment uses electricity and argon gas in combination with other bronchoscopic treatments to kill tumor tissue.

• Rigid Coring

  This palliative procedure involves inserting a rigid bronchoscope into the trachea and pushing it through the center of the tumor to open up the airway. Rigid coring, which is frequently used in combination with other bronchoscopic treatments, can be highly effective.

Tumors in the major bronchi can be managed with similar techniques, including surgical resection, airway stent placement, and bronchoscopic treatments.

Radiation Therapy

Radiation therapy may be used to treat patients with a tumor that involves more than 50 percent of the trachea, or has spread to nearby lymph nodes or other areas within the chest, and to treat patients who are in poor general health. Some patients with adenoid cystic tumors may be treated with radiation therapy alone or following surgery. External-beam radiation, in which a beam of radiation is delivered from an external source to the tumor site, is the main type of radiation therapy used to treat patients with tracheal tumors. Brachytherapy — a localized form of radiation therapy — may be used to treat patients who are not candidates for external-beam radiation therapy.