Tracheal adenoid cystic carcinoma

Case contributed by Brian Gilcrease-Garcia
Diagnosis certain

Presentation

Hemoptysis. Lifelong history of smoking.

Patient Data

Age: 50 years
Gender: Female
  • irregular circumferential thickening of mid to distal tracheal and extending into left mainstem bronchus, with more mass-like component at the level of carina
  • the tracheobronchial lesion causes severe airway narrowing, and the left lung has a diffusely lucent appearance, suggestive of mild air trapping
  • no evidence of involvement of other adjacent mediastinal structures, including esophagus or greater vasculature. No lymphadenopathy or suspicious pulmonary nodule
  • Diffuse ground glass opacities of the right lower lung are nonspecific and may indicate atelectasis

Bronchoscopy

Photo

Exophytic, somewhat polypoid mass originating from the tracheal wall. It extends in craniocaudal length from mid thoracic trachea down into left mainstem bronchus. Biopsy was obtained.

Case Discussion

Imaging findings of tracheal wall thickening may be considered according to pattern of focal vs diffuse involvement of the upper airway (see article for more). The presenting symptom of hemoptysis is nonspecific, as it may occur with infectious/inflammatory etiologies or neoplasia. In this case, the circumferential irregular tracheal thickening on CT has a mass-like appearance (particularly at the carina), most consistent with tumor. As the lesion is centered in the trachea and there is no other known malignancy, the main diagnostic consideration is primary tracheal tumor.

Although primary tumors of trachea are rare, they most commonly arise from epithelial cells (squamous cell carcinoma) or salivary glands (adenoid cystic, mucoepidermoid carcinomas). Adenoid cystic carcinomas were previously known as "cylindromas" due to their circumferential growth pattern and resulting cylindrical appearance by imaging.

 

Histopathology

Trachea, "endobronchial lesion," biopsy

The tumor predominantly shows a cribriform pattern, consistent with a Grade 2 adenoid cystic carcinoma in this biopsy material, which may not be representative of the entire tumor.

Diagnosis: Adenoid cystic carcinoma

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