Tracheal Tumors Clinical Presentation

Updated: Sep 23, 2019
  • Author: Brian J Daley, MD, MBA, FACS, FCCP, CNSC; Chief Editor: Mary C Mancini, MD, PhD, MMM  more...
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Presentation

History and Physical Examination

The presentation of primary tumors of the trachea is variable. Usually insidious in onset, tracheal tumors often present with signs and symptoms of upper airway obstruction. In a series of 329 patients with primary tracheal malignancies, dyspnea was the most frequent symptom (71%), followed by cough (40%), hemoptysis (34%), asthma (19.5%), and stridor (17.5%). [3] These symptoms are often misinterpreted, leading to a delay in diagnosis and a trial of corticosteroids before the correct diagnosis is made. Symptoms related to involvement of adjacent structures, such as hoarseness and dysphagia, are less common (7%).

The first symptom may be shortness of breath after activity, which gradually worsens. Acute respiratory difficulty may not be present until the airway is almost completely occluded, which explains why it may be rapidly fatal. A persistent cough, wheezing, or stridor may be noted, as may recurrent attacks of respiratory obstruction caused by secretions.

Delay in diagnosis occurs because the pulmonary fields remain normal on a chest radiograph. [3] If the patient has hemoptysis, a diagnosis is more likely to be made because bronchoscopy will be performed even in the presence of a normal chest radiograph. This is more common in patients with squamous cell tumors.

Patients may also present with repeated episodes of either unilateral or bilateral pneumonia that respond to antibiotics and physiotherapy. In the absence of hemoptysis, a diagnosis of adult-onset asthma is often made, thus delaying definitive treatment. In one series, delayed diagnosis of more than 6 months after symptoms onset occurred in one third of patients.

Squamous cell carcinoma (SCC) is the most rapid in onset, often leading to hemoptysis or obstructive symptoms as presenting features. It is more prone to be exophytic and ulcerative in nature and is most common in male smokers. SCC metastasizes to regional lymph nodes and can invade the mediastinum in more aggressive forms or in late stages. Distant metastases are common, most often to bone.

Adenoid cystic carcinoma (ACC), or “cylindroma,” occurs almost as frequently as SCC; together, the two types account for two thirds of primary tracheal malignancies. ACC is slower in onset than SCC, often having a prolonged course of clinical symptoms. ACC may extend over long distances in the submucosa. It too spreads to regional lymph nodes, though less often than SCC does. ACC often displaces mediastinal structures in late stages before actually invading them. Metastases to the lung are not uncommon. The male-to-female ratio is almost equal, and ACC is less commonly associated with smoking.

One quarter of tracheal tumors are neither ACC nor SCC. This heterogeneous group of tumors has varying degrees of malignancy and include both epithelial and mesenchymal histotypes. In a review over 40 years, Gaissert et al assessed 360 tumors and documented 90 unusual tumors. [7] Unusual tumor types included the following:

  • Carcinoid (11)
  • Mucoepidermoid (13)
  • Nonsquamous bronchogenic carcinoma (15)
  • Lymphoma (2)
  • Melanoma (1)

Thirty-four lesions were benign.