Adenoid cystic carcinoma - airway

Case contributed by Liz Silverstone
Diagnosis certain

Presentation

Stridor.

Patient Data

Age: 30 years
Gender: Female

Convex bulging of the right superior mediastinal border.

Narrowing of the trachea at the level of the aortic arch.

Carina poorly demarcated.

Otherwise normal.

Narrowing of the trachea, carina and proximal main bronchi associated with circumferential wall thickening displacing the SVC and separating the trachea from the aortic arch.

Small polypoid protrusion arising from the posterior wall at the carina.

DIAGNOSTIC SUMMARY: Distal tracheal tumor biopsies: Adenoid cystic carcinoma.

Macroscopic Description: Labeled "biopsy distal tracheal tumor", the specimen consists of four tan fragments of tissue 5 to 11mm. All embedded.

Microscopic Description: Distal tracheal tumor. These are biopsies of adenoid cystic carcinoma; some normal respiratory epithelium is present on the surface of some biopsies. The tumor has typical morphology; there are no high grade features. The tumor is negative for HER2, androgen receptor and PSMA by immunohistochemistry. Additional immunohistochemistry: Bcl2 is positive with strong (3+) and diffuse pattern.

Case Discussion

Adenoid cystic carcinoma of the tracheobronchial tree is a rare disease and was treated by complete surgical excision and adjuvant radiotherapy 1,2. Residual right main bronchial narrowing was relieved by a metal stent. Recurrent restenosis due to ingrowth of granulation tissue necessitated repeated stent replacement.

Major airways are an important part of the systematic review of CXRs and should be scrutinised in patients presenting with stridor.

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