The clinical and pathologic features of carcinoma arising in Barrett's esophagus were studied in resection specimens from 26 patients. White males predominated (73%). A history of symptomatic gastroesophageal reflux was frequently absent, being elicited in only eight of 14 patients (57%) with a carefully obtained history at the time of presentation with carcinoma. Survival was relatively short with a median survival of 23 +/- 5 months, and only three patients had a disease-free survival longer than 2 years. A pathologic spectrum of carcinoma was found: differentiation ranged from well to poorly differentiated in the 20 patients with a single adenocarcinoma; two separate carcinomas were found in four patients; and a spectrum of differentiation in a single tumor was found in the other two cases, one an adenocarcinoid tumor and the other an adenosquamous carcinoma. The tumors were generally far advanced, with extension through the esophageal wall in 23 of 26 cases (88%) and metastases to lymph nodes in 17 of 24 cases (71%). Epithelial dysplasia, including carcinoma in situ in some cases, was found in Barrett's mucosa adjacent to the tumor in all 26 patients. Our findings suggest that a surveillance program for dysplasia in patients known to have Barrett's esophagus is warranted in an attempt to improve the outcome. However, the impact of surveillance on the incidence of Barrett's carcinoma may be lessened by its frequent occurrence in patients with asymptomatic gastroesophageal reflux.