Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.

Primary Malignant Tracheal Tumors
Michael G. Stewart, M.D.
January 4, 1992

The average adult trachea is 11 cm in length, and there are approximately 2 cartilaginous rings per cm. The blood supply to the upper trachea is primarily from branches of the inferior thyroid arteries, and to the lower trachea from branches of the bronchial arteries with contributions from the supreme intercostal, internal thoracic, innominate and subclavian arteries. The blood supply enters the trachea laterally.

Primary tracheal tumors are very rare, about 0.1 per 100,000 population, and over 90% are malignant. The incidence of tracheal carcinoma is much lower than laryngeal or endobronchial cancer.

There are two main histologic types of primary tracheal carcinoma: squamous cell CA, and adenoid cystic CA. The next most common primary malignant tumors were carcinoid tumors and mucoepidermoid carcinoma. Approximately 10% of primary tumors were benign. The most common benign tumor was squamous papilloma, and other types included pleomorphic adenoma, granular cell tumor, and benign cartilaginous tumors.

Most secondary tracheal tumors are caused by direct extension of a local tumor into the trachea, such as papillary or follicular thyroid carcinoma, aggressive lung cancer, or esophageal carcinoma. In addition, there are rare reports of distant metastatic disease to the trachea.

The incidence of multiple primary carcinomas in tracheal carcinoma is high. In the M.D. Anderson series, the incidence of multiple primaries was 28%, which is similar to other published series.

The most common symptoms and signs at presentation are hemoptysis, cough, dyspnea on exertion and wheezing or stridor. Dysphagia and hoarseness are less common at presentation, and are both bad prognostic signs. Hemoptysis is more common in patients with squamous cell carcinoma, and usually leads to earlier diagnosis. Adenoid cystic carcinoma often presents with wheezing or stridor, leading to a delay in diagnosis.

Endoscopy and radiography are the usual methods of diagnosis of tracheal tumors. Submucosal spread is common in adenoid cystic carcinoma, and biopsies of clinically normal trachea at sites distant from the tumor are recommended.

Tracheal tumors are notoriously difficult to diagnose on plain chest X-ray. Tracheal tumors were identified in 21 of 22 patients who underwent tracheal tomography, however. CT scanning gives the best estimation of mediastinal involvement.

The two main histologic types of primary tracheal malignancy have different natural histories. Squamous cell carcinoma may be ulcerative or exophytic in appearance. It tends to metastasize to regional lymph nodes, and often directly invades mediastinal structures. Second primary squamous cell malignancies are common. Locoregional recurrence is common and is usually fatal within months. The five-year survival after curative resection is historically reported as 20 to 40%. Recently Grillo reported 49% survival, with some patients not followed five years.

Adenoid cystic carcinoma has a more insidious onset, and a much slower growth pattern. Tumors may involve a large length of trachea by submucosal spread without involving adjacent mediastinal structures. Often the mediastinal structures are "pushed" aside, rather than invaded directly by tumor. Only about 10% of adenoid cystics will metastasize to regional lymph nodes, although hematogenous metastases--especially to the lung--are not uncommon (35% in one series). Perineural growth is commonly seen. Although local recurrence after resection is very common, it may not present clinically for five or ten or more years. The five-year survival after resection is reported as 60% to 100% (Grillo reports 75%), and patients may survive for many years with microscopic or metastatic disease, but many will recur locally or metastasize, and patients often die of disease.

For upper tracheal tumors, a cervical collar incision is recommended. If the middle or lower trachea is involved, Grillo recommends right posterolateral thoracotomy, while Pearson, et al, recommend midline sternotomy and a transpericardial approach. The blood supply to the trachea travels in lateral tissue pedicles, and these should be carefully preserved. Experimental studies have demonstrated that a tension-free suture line is most important in a successful anastomosis. Tracheal mobilization is necessary to achieve a tension-free anastomosis. Dissection of the anterior pretracheal tissue planes, while preserving the lateral blood supply, permits some tracheal mobilization. Grillo and Mathisen report that simple neck flexion is the most useful single maneuver for achieving primary repair.

There are several additional mobilization techniques available. Perhaps the most popular is the suprahyoid laryngeal release. An infrahyoid release is also described. The distal trachea may be mobilized by mobilization of the right pulmonary hilum, and the left mainstem bronchus may be divided and reanastomosed end-to-side with the bronchus intermedius.

If the amount of trachea involved is greater than 50 to 60%, primary repair is usually not possible. Marlex and Silicone grafts are described. The major problems with synthetic grafts are the rigidity of the material used, and the failure of ciliated respiratory mucosa to cover the lumen of the graft, leading to difficulty clearing respiratory secretions.

In cases of airway compromise and unresectable disease, the airway may be maintained by repeated bronchoscopic debulkings, use of the CO2 laser, the silicone Montgomery T-tube, and tracheal stents.

Both adenoid cystic and squamous cell carcinoma are usually radiosensitive, and radiotherapy has been utilized as both a primary and an adjunct treatment for primary tracheal carcinoma. Most authors recommend primary surgical excision, if possible, with post-operative XRT. In unresectable disease, some palliation may be achieved with XRT. Pre-operative XRT is not recommended.

Case Presentation

The patient is a 63-year-old white male with a long smoking history, who presented with a two month history of sore throat and hemoptysis. His medical history was significant for a right lower lobectomy in 1988 for adenocarcinoma. Physical examination revealed a T3N2A squamous cell carcinoma of the pyriform sinus. During his pre-operative evaluation for total laryngectomy, a chest CT scan revealed a large paratracheal mass at the level of the carina. Bronchoscopy revealed an exophytic mass in the lower trachea arising from the posterior wall and occluding 50 to 60% of the lumen. Biopsy revealed squamous cell carcinoma.

Because of the extratracheal extension of tumor, the patient was not felt to be a candidate for tracheal resection. He is currently receiving a combination chemotherapy and radiotherapy protocol to both his tracheal and pyriform sinus lesions.

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Last modified: Jan. 23, 2006