A case of primary adenoid cystic carcinoma of the esophagus is reported and a total of 15 cases was reviewed. There were 9 men, although earlier reports indicated the disease was more common in women. The lesion was located in the middle third of the esophagus in 10 cases. The possibility of tumor development from embryonal rests of the tracheobronchial tree in the esophagus is discussed. The prognosis of adenoid cystic carcinomas is not well known, but the treatment and prognosis appear similar to those of epidermoid carcinomas of the esophagus.

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