Adenoid cystic carcinoma of the trachea

Abstract

Primary salivary gland-type tumors of the lung and airways being unusual, they pose a diagnostic challenge on small biopsies and are usually consigned as non-small cell lung carcinomas. Since the clinical behavior of these tumors is different from the conventional lung tumors, it is important to accurately diagnose them. Among this category of tumors, adenoid cystic carcinoma (ACC) of the lungs and airways is exceedingly rare. Few large studies and maximum case reports have been reported in literature on ACC of the lungs and trachea so far. We herein report a rare case of primary ACC of the trachea diagnosed on bronchoscopic biopsy.

A Delicate Surgical Resection to Treat a Proximal Pulmonary Adenoid Cystic Carcinoma

“…So far, several large studies and more case reports have been reported in the literature about PACC. This tumor is unrelated to smoking, it appears usually in the fifth decade, and there is no particular sex predilection [ 2 , 4 , 5 ]. Our patient was, so, younger than usually reported.…”

“…Most ACC show a pattern mixture. Immunostaining is not necessary for the diagnosis but can serve out in several special cases notably in solid or tubular ACCs [ 2 ]. Tumor cells express smooth muscle actin and myosin, the protein S100, and CD117 [ 8 ].…”

“…It shows up in CT scan as an intraluminal mass extending through the tracheal wall. Sometimes, the lesion may present as a circumferential parietal thickening [ 2 , 9 ]. Respiratory functional assessment is mandatory after the diagnosis of ACC.…”

“…Diagnosis is often late because the symptoms appear only after the tumor infiltrates the proximal airways. Symptoms are usually noncharacteristic, starting with cough and leading to dyspnea on exertion [ 2 ]. PACC has an unpredictable growth behavior.…”

A SEER database cohort of 868 patients with primary tracheal cancers: characteristics and outcomes and the role of bronchoscopic interventions

“…Primary tracheal tumors are overwhelmingly malignant in adult patients and usually benign in children [21][22][23] . Squamous cell carcinoma (SCC) and adenoid cystic carcinoma (ACC) are the 2 most common histologies and represent about two thirds of these tumors in adults [15,21,24,25] . They are followed by other rarer neoplasms such as mucoepidermoid carcinoma, sarcomas, lymphomas, and melanomas [17] .…”