Key Points

• The most common cause of malignant tracheal obstruction—metastatic disease—must be considered carefully before the diagnosis of a primary tumor is made.
• Expect a delay in diagnosis whenever a primary tracheal tumor is evaluated and do not assume that this delay indicates unresectable disease.
• Evaluation by bronchoscopy is possible in every patient with a tumor of the trachea or carina and provides the most useful information.
• Balance the benefit of complete resection with negative airway margins against the risk of excessive tension at the anastomosis. If in doubt, decide in favor of a secure anastomosis.

In comparison to the common malignancies of larynx and lung, primary tumors of the trachea are rare. Malignant tracheal obstruction is caused far more often by metastatic than primary tumors. Surprising is therefore not the frequent delay in diagnosis and therapy but the high proportion of resectable primary tumors encountered in centers for airway surgery. Resection may be offered, at low operative risk in experienced hands, to a majority of patients with tracheal tumors, but the dismal survival and low resection rates observed in epidemiologic studies suggest that, other than at centers for tracheal surgery, resection is considered in very few patients. The establishment of regional referral centers has been proposed for this purpose. However, with increasing awareness of surgical techniques and nonsurgical treatment, the experience with this rare tumor is likely to scatter. In this chapter we summarize the current knowledge of the incidence, evaluation, and management of these tumors.