Tracheal Cancer (Cancer of the Windpipe)

What tracheal cancer is

The trachea is a tube about 10 to 12 cm (4 to 5 inches) long, held open by C-shaped rings of cartilage. It runs from the larynx (voice box) in the neck down into the chest, where it splits into the two main bronchi. Its lining contains surface cells and small mucus glands.

Primary tracheal cancer starts in the trachea itself and is rare. Much more often, cancer reaches the trachea by spreading from nearby organs: the lungs, larynx, esophagus or thyroid. These are treated as cancers of their organ of origin.

Types

Squamous cell and adenoid cystic carcinoma together make up about two-thirds of primary tracheal cancers. Benign tracheal tumors, such as papillomas and chondromas, also occur.

Symptoms

Symptoms usually appear only when the tumor has narrowed the airway by more than half, because the trachea is wide:

• Shortness of breath, first on exertion and later at rest
• Wheezing that does not improve with asthma inhalers
• Stridor: a high-pitched, harsh sound when breathing in
• Persistent cough
• Coughing up blood (hemoptysis), more typical of squamous cell carcinoma
• Hoarseness if the tumor affects the nerves to the voice box
• Difficulty swallowing if it presses on the esophagus
• Repeated chest infections or pneumonia
• Weight loss and tiredness

Because these symptoms suggest asthma or COPD, many people are treated for those conditions for months before the cancer is found. Breathlessness that does not respond to inhalers, especially with noisy breathing on breathing in, warrants further investigation.

Causes and risk factors

• Smoking: the main risk factor for squamous cell carcinoma
• Age: most squamous cell cancers occur after 50
• Previous head, neck or lung cancer, or radiation to the chest
• Adenoid cystic carcinoma has no known lifestyle cause

Diagnosis

1. CT scan of the neck and chest: shows the tumor, how long a section of trachea is involved, and spread to lymph nodes or nearby structures.
2. Bronchoscopy: a thin camera passed through the nose or mouth into the airway to see the tumor and take a biopsy. Rigid bronchoscopy under general anesthesia also allows urgent removal of blocking tumor.
3. Lung function tests: a flattened flow-volume loop is a classic sign of a fixed upper airway blockage.
4. PET-CT: to check for spread elsewhere.
5. Assessment by a thoracic surgery team to decide if the tumor can be removed.

Treatment

Surgery

Tracheal resection and reconstruction removes the cancerous segment and joins the two healthy ends together. Up to about half of the adult trachea can be removed and reconnected in experienced centers. Surgery gives the best chance of long-term survival for both main types. Because these operations are rare and complex, they are best done at specialist centers.

Radiation therapy

• After surgery, especially if the cut edges contain cancer cells, which is common with adenoid cystic carcinoma
• As the main treatment when surgery is not possible
• Brachytherapy (internal radiation placed in the airway) in selected cases

Chemotherapy and targeted therapy

Used together with radiation for some squamous cell cancers, and for cancer that has spread. Clinical trials are an option for advanced disease.

Relieving airway blockage

When the airway is critically narrowed, doctors can open it with bronchoscopic techniques: removing tumor with a rigid scope, laser or heat therapy, cryotherapy, or placing a stent. These relieve breathlessness quickly and can be used before definitive treatment or for comfort in advanced cancer.

Outlook

Outlook depends mainly on tumor type and whether it can be completely removed:

• Adenoid cystic carcinoma: grows slowly, and after surgery, most people are alive at 5 years, with many living much longer. Late recurrence is possible, so long-term follow-up is needed.
• Squamous cell carcinoma: a less favorable outlook, but people whose tumors are completely removed do much better than those who cannot have surgery.

Stopping smoking improves surgical recovery and lowers the risk of a second cancer. Follow-up includes regular CT scans and bronchoscopy.

Frequently asked questions

What are the first signs of tracheal cancer?

Early signs are often shortness of breath, wheezing, a persistent cough and sometimes coughing up blood. Because they mimic asthma or bronchitis, they are easy to overlook. Wheezing or breathlessness that does not improve with inhalers, or noisy breathing on breathing in, needs investigation.

How rare is tracheal cancer?

Very rare. Primary tracheal cancer affects roughly 1 person per million each year and accounts for well under 1% of cancers. Cancers that spread to the trachea from the lungs, esophagus or thyroid are more common than cancers starting there.

What is the survival rate for tracheal cancer?

It varies by type and treatment. People with adenoid cystic carcinoma that is surgically removed often live many years, and most are alive at 5 years. Squamous cell carcinoma has a lower survival rate, but complete surgical removal greatly improves the outlook. Your care team can give figures specific to your situation.

Is tracheal cancer caused by smoking?

Smoking is the main cause of squamous cell carcinoma of the trachea. However, adenoid cystic carcinoma, the second most common type, is not linked to smoking and often affects people who have never smoked. Anyone with persistent airway symptoms should be checked, whatever their smoking history.

Can you live without part of your trachea?

Yes. Surgeons can remove a section of trachea, often up to about half its length in adults, and join the ends together. After healing, most people breathe and speak normally. In the first days, the chin may be kept tilted down to reduce tension on the join.