We report a case of a 64-year-old Japanese man with adenoid cystic carcinoma. An elevated lesion covered by intact epithelium in the thoracic esophagus was found in September, 2007 and been followed. After dysphagia appeared follow-up endoscopy was performed in January, 2010, and morphological change into a protruding tumor was recognized. Since adenoid cystic carcinoma was detected by endoscopic biopsy, the patient underwent esophageal resection. The resected specimen showed a cribriform pattern and a certain amount of mucous substance which was positive for Alcian blue, within a solid nest. The tumor cells were positive for S-100 protein and negative for αSMA, so the tumor was diagnosed as an adenoid cystic carcinoma.

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