Adenoid cystic carcinoma of the trachea: A report of seven cases and literature review

Background: Adenoid cystic carcinoma (ACC) of the trachea is rare. The clinical manifestations, natural history, and responses to therapy differ from those of other malignant tracheal tumors. Method: In this retrospective study, we examined pathological records of patients in the Department of Pathology at Chang Gung Memorial Hospital from 1990 through 2002. There were five male patients and two female patients with ages that ranged from 21 to 55 years. For these seven patients the clinical manifestations and period from initial symptoms to diagnosis, treatment and outcome are presented. Results: Hemoptysis, nonproductive cough, dyspnea, chest pain and weight loss were the frequent early manifestations. Five patients had tumors in the lower area of the trachea, two had tumors in the upper area of the trachea. In a patient with the tumor in the upper area of the trachea, the tumor involved the cricoid ring, larynx, and subglottic area. In one patient with the tumor in the lower area of the trachea, the tumor invaded the right lower lobe of the lung. The longest survival time was more than 10 years. Conclusions: ACC is a rare primary tracheal malignancy. The time from first symptoms to diagnosis varied, ranging from weeks to more than 1 year. Complete surgical resection provides the patient with the best chance of prolonged survival or even complete remission.