ETIOLOGY
Etiology—Key Facts
Squamous cell carcinoma and adenoid cystic carcinoma compose the majority of malignant tracheal neoplasms
Malignant
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Squamous cell carcinoma
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Approximately 40% of malignant tracheal neoplasms
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Can present as either an exophitic or ulcerative lesion and may occur anywhere in the trachea or bronchial tree
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May invade adjacent structures (recurrent laryngeal nerve/esophagus), but distant metastasis are rare early in presentation
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Associated with tobacco abuse
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Adenoid cystic carcinoma
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Approximately 40% of malignant tracheal neoplasms
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Formally known as a cylindroma
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May present as a mass in the trachea but may extend significant distances within the submucosa
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Extremely radiosensitive, and positive margins following surgery are treated with postoperative radiotherapy
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Can present in any age range and not associated with smoking
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Other primary malignant tracheal masses (rare)
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Adenocarcinoma
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Adenosquamous carcinoma
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Small cell carcinoma
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Basaloid squamous cell carcinoma
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Atypical carcinoid
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Malignant fibrous histiocytoma
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Melanoma
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Chondrosarcoma
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Spindle cell sarcoma
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Rhabdomyosarcoma
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Fibrosarcoma
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Leiomyosarcoma
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Kaposi’s sarcoma
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Lymphoma
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Lymphoepithelial carcinoma
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Angiosarcoma
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Invasive thyroid carcinoma
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Invasive esophageal carcinoma
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Benign
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Benign squamous papilloma
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Multiple solitary pleomorphic adenoma
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Granular cell tumor (myoblastoma)
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Glomus tumor
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Fibroma
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Fibrous histiocytoma (pseudotumor, plasma cell granuloma, xanthoma)
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Lipoma
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Leiomyoma
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Hamartoma
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Chondroma
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Chondroblastoma
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Schwannoma
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Neurofibroma
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Paraganglioma
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Hemangioma
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Hemangioendothelioma
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Vascular malformation
CLINICAL FEATURES
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Symptoms and Signs:
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Initially, patients often present with a long history of coughing progressing to wheezing. They are frequently treated for adult onset asthma or chronic obstructive pulmonary disease before the diagnosis of a tracheal mass.
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Wheezing can progress to stridor as the caliber of the airway continues to narrow. Chest radiographs will often continue to demonstrate clear lung fields. Chest tomograms will occasionally reveal the tracheal mass.
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Hemoptysis is a late finding and more often occurs with squamous cell carcinoma than adenoid cystic carcinoma.
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With low lesions abutting the carina, postobstructive pneumonia can occur and needs to be evaluated with bronchoscopy
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Late findings in the course of tracheal tumors are increasing hoarseness from invasion of the recurrent laryngeal nerves and dysphagia from esophageal invasion or compression.
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DIAGNOSIS
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History
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Important components of the history include
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Cough
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Progressive shortness of breath
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Progressive dyspnea
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Wheezing unresponsive to bronchodilators
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Hemoptysis
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Recurrent pneumonia/pneumonitis
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Change in voice/increasing hoarseness
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Dysphagia
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Thoracic/cervical malignancies
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Lung
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Esophageal
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Thyroid
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Parathyroid
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Laryngeal
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Squamous cell carcinoma of the head and neck
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Carcinoid tumor
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Physical examination
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Confirm airway patency
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Ensure that trachea is midline
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Bilateral breath sounds
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Pulmonary auscultation
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Tracheal auscultation
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Head and neck exam including:
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Thyroid examination
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Cervical lymph nodes, masses
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Palpate tracheal rings, thyroid and cricoid cartilages
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Jugular venous distention
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Oral and pharyngeal exam
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Radiographic evaluation:
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Plain chest radiograph
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Normal in majority of patients
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Often leads to continuation of current therapy (i.e., bronchodilators), rather than guiding intervention to look for tracheal mass
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Helical computed tomography (CT) scan
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Occasionally will demonstrate a tracheal mass. Addition of high-resolution CT images with thin cuts and three-dimensional and multiplanar two-dimensional reconstructions increases sensitivity and specificity ( Fig. 13-1 )
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