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.Key Words: salivary-type tumors of lung and bronchus, adenoid cystic carcinoma, mucoepidermoid carcinoma (Pathology Case Reviews 2004;9: 253-258)
CASE REPORTA 38-year-old woman, a nonsmoker, presented initially with complaints of chronic cough of 3 years duration. In the course of her evaluation, imaging revealed a 5 ϫ 6 cm right upper lobe lung mass involving the right mainstem bronchus. Bronchial brushings and washings demonstrated scattered groups of atypical mucinous-type cells, few papillary clusters of epithelial cells with rare groups of atypical cells, and few groups of markedly atypical cells. A bronchial biopsy and second bronchial brushing were both interpreted as adenoid cystic carcinoma (ACC; Fig. 1).A right total pneumonectomy was performed, and gross examination demonstrated a 4 ϫ 4 cm gray-white solid tumor in the right upper lobe involving the bronchus and abutting but not extending through the pleura. An intraoperative frozen section of the bronchial margin was positive for ACC, and reexcision of additional right upper lobe tissue and bronchus was negative for tumor on the final permanent sections. Eleven regional lymph nodes were also negative for tumor.Microscopic examination showed tumor surrounding the bronchus, extending and infiltrating into the pulmonary parenchyma (Fig. 2). Variable architectural patterns included cribriform areas and solid nests of basaloid-appearing cells with intervening collagenous stroma. The cribriform areas contained 2 types of spaces: true duct-like spaces lined by cuboidal cells with eosinophilic cytoplasm and so-called pseudocystic spaces with no true ductal lining cells. The latter consisted of cylindric spaces surrounded by cells with amphophilic cytoplasm and oval to angular nuclei. Some of the pseudocystic spaces were filled with eosinophilic basement membrane-like material, and others contained slightly basophilic mucinous-appearing material. Extensive perineural involvement and lymphovascular space invasion were identified. Mitotic figures were rare, and necrosis was absent.The patient received postoperative radiation treatment. After a 7-year ...