Adenoid Cystic Carcinoma of the Lung

Abstract

Primary adenoid cystic carcinoma of lung (ACCL) represents one of a group of salivary-type tumors of lung, likely originating from submucosal seromucinous glands in the pulmonary bronchial tree. Although it comprises less than 1% of all primary lung carcinomas, ACCL is considered the second most common primary salivary-type tumor of lung, after mucoepidermoid carcinoma. Typically, patients with ACCL present at a younger age than those with other primary lung carcinomas, although the age range is broad and ranges from the second to eighth decades. ACCL shares histologic and immunophenotypic features with ACC of salivary gland location, and exhibits a similar infiltrative growth pattern with a propensity for perineural invasion. Treatment is primarily surgical, and therefore complete surgical resection, with negative bronchial surgical margins at intraoperative frozen section, is important for the possibility of cure.