Tracheal tumors tend to cause progressive symptoms over several months to years due to their slow-growing nature often causing accurate diagnosis to be delayed (6). Gaissert et al. (7) reviewed 270 cases of squamous cell and adenoid cystic carcinoma of the trachea and carina, and the mean duration of symptoms was 12.2 months. The duration of symptoms was longer in adenoid cystic carcinoma and in tumors that were deemed to be unresectable (resected adenoid cystic carcinoma 18.3 months, resected squamous cell carcinoma 4.54 months, unresectable adenoid cystic carcinoma 23.7 months, unresectable squamous cell carcinoma 7.58 months, P < 0.0001) (6).

Symptoms at presentation depend largely on extent of airway obstruction and tumor type, but not location within the tracheobronchial tree (8). Dyspnea with exertion or phonation is the primary symptom when the tumor has caused significant airway obstruction. If lesions are ulcerative and mucosal irritation is severe, chronic cough and hemoptysis are common. Wheezing, stridor, hoarseness, difficulty clearing secretions, and frequent respiratory infections are also encountered.

Presentation may vary with tumor type with hemoptysis being the main symptom in patients with squamous cell carcinoma, and wheezing and dyspnea predominate in those with adenoid cystic carcinoma (Table 126.2) (9). Wheezing is often misdiagnosed as “asthma” and is refractory to bronchodilator therapy. Prolonged use of systemic corticosteroids is common, and patients may even be secondarily cushingoid at presentation (10).

Imaging studies that are most frequently employed are CT scan with contrast and in some instances PET-CT. CT with contrast allows for adequate multiplanar imaging of the airway from hyoid to segmental bronchi. CT can be further exploited to derive three-dimensional images allowing for “virtual endoscopy.” It is useful in the identification of involvement of mediastinal soft tissues, esophagus, and vasculature. It is additionally helpful in demonstrating coexisting pulmonary metastatic disease. PET-CT may be useful in distinguishing more aggressive pathology and may be more sensitive in the detection of paratracheal and mediastinal nodal involvement. MRI scanning may be useful in the examination of the cervical trachea, but mediastinal and thoracic structures may be significantly be distorted by motion artifact due to respiratory and circulatory movement (11,12,13). Figure 126.1A and B represent a demonstration of a high tracheal adenoid cystic carcinoma in both the axial and coronal planes.

Tracheal resection and primary reanastamosis is the preferred treatment for the majority of both benign and malignant tracheal neoplasms. The ability to perform this preferred management is predicated upon patient-, tumor-, and surgeon-related factors. Patient factors that are important include body habitus, medical comorbidities, and patient preference. Increasing body mass index (BMI) and a thick and short neck may be impediments to the execution of tracheal resection and reanastamosis. In this patient group, the mobilization of the trachea inferiorly and superiorly may be limited, and access to the segment of trachea containing the tumor may not be able to be accomplished solely through a cervical approach. Patients with coexisting chronic obstructive pulmonary disease may require a longer intubation and may be more prone to coughing exacerbations that may place additional strain on the tracheal repair. Furthermore, patients who have diabetes mellitus or are otherwise immunocompromised may have poorer wound healing, especially with longer segment resections that are under tension.

Tumor factors are important in decision making. The length of the proposed segment that would require resection for adequate margins represents one of the key factors in determination of treatment. Segmental resections of greater than 4 cm or six tracheal rings represent conditions in which the tracheal repair is under increased tension and dehiscence may be more likely. Submucosal, esophageal, mediastinal, and extensive tracheal or bronchial extension may require combined cervicothoracic procedures or nonsurgical treatment. A variety of extended tracheobronchial resections have been described and are best managed in a multidisciplinary approach with otolaryngologist and thoracic surgeon. Premature tracheotomy in the securing of an airway may create additional scar tissue and potential seeding of the soft tissues of the neck and make subsequent surgical resection efforts more difficult. Prior radiation therapy may additionally have an impact on the ability to dissect soft tissues, mobilize the trachea and larynx, identify critical structures, and allow for optimal wound healing.

Surgeon preference and experience may influence approach. Experience with benign tracheal stenosis, laryngectomy, and tracheal resection increases the confidence and ability of the surgeon to manage these lesions. Cooperation between otolaryngologists and thoracic surgeons allows for improved patient selection, procedure selection, and technical execution of these complicated cases (2,3,4,5,15). Contraindications for tracheal resection are relative rather than absolute in many cases. Because airway patency represents a fundamental goal in the management of patients with tracheal neoplasms, resection may be offered even in the face of primary tracheal tumors with regional or distant metastatic disease as well as selected metastatic lesions to the trachea. Lesions
that are involve greater than 4 cm or are greater than six tracheal rings may not be eligible for resection; factors ultimately affecting this decision include body habitus, ability to adequately mobilize the upper respiratory tract, and experience of the surgical team in both the procedure and management of its complications. Extensive extratracheal disease in the mediastinum involving the esophagus, mediastinal vessels, and prevertebral spaces represents cases in which resection is contraindicated and tracheotomy, endoscopic tumor ablation, and stent placement may be preferred.

Standard resection steps are predicated upon adequate bronchoscopic evaluation of the airway, imaging of the trachea and related neck and mediastinal structures, pathological confirmation, surgical team selection and approach, anesthetic preparation and management plan, and finally execution of the procedure. The steps of the tracheal resection procedure are as follows: transverse cervical incision outlined in cervical crease at midpoint between the sternal notch and the inferior border of the cricoid cartilage that are readily palpable landmarks. In extended cases or in cases in which cervical and extensive mediastinal dissection or proximity to the innominate artery is anticipated, both the neck and chest should be sterilely prepped and draped. The transverse cervical incision can be readily connected to a vertical chest incision for addition of a median sternotomy (total or partial). After preparation and skin incision choices are made, the steps of tracheal resection with primary repair are as follows: