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Airway neoplasms account for approximately 90% of carinal resections.1 The incidence of primary tracheal tumors is unclear, but is known to be rare. A recent population-based cancer registry analysis using the SEER database demonstrated an incidence of 2.6 tracheal tumor cases per 1,000,000 people per year.2 Carinal tumors, as a subcategory of tracheal tumors, are even less common. Most are malignant and can be divided into bronchogenic carcinoma and other airway neoplasms. Bronchogenic carcinomas are by definition malignant; the other airway neoplasms may exhibit a wide range of behavior. As demonstrated in Table 64-1, the most common malignant primary tracheal neoplasms are squamous cell carcinoma (SCC) and adenoid cystic carcinoma (ACC).3 SCC occurs primarily in smokers in their sixth and seventh decades and may present confined to the trachea or invading into adjacent mediastinal structures. ACC is an exophytic intratracheal lesion, which may involve the tracheal wall to variable extent (Fig. 64-1), and compress mediastinal structures without invading them initially. Lymph node metastases occur, but less commonly than in SCC. A characteristic feature of ACC is its proclivity for extending long distances submucosally and perineurally.

Table 64-1Histologic Types of Carinal Neoplasms from A Single Institution 1962 to 1996
Figure 64-1

Adenoid cystic carcinoma invading through the anterior carinal wall into mediastinal space and abuts the superior vena cava.

Clinical Presentation

Patients commonly present with symptoms and signs of central airway obstruction. They have worsening dyspnea, often progressing to wheezing and/or stridor as the diameter of the airway decreases. Dyspnea on exertion occurs when the airway diameter is less than 8 mm and stridor develops with airways less than 5 mm.4 Chest radiographs may demonstrate a mass in the tracheobronchial airway column. These findings are often subtle and are usually missed. Consequently, patients are commonly given a diagnosis of adult-onset asthma, and diagnosis is delayed. Patients presenting with either postobstructive pneumonia or a cough with hemoptysis may have their tumors diagnosed more rapidly. Extensive tumors may result in hoarseness, dysphagia or chest discomfort suggesting more diffuse or extensive mediastinal invasion.

Diagnostic and Staging ...

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