Several case reports have emphasized that esophageal
carcinoid tumors are associated with a poor prognosis. To expand our knowledge about the pathology and
biologic behavior of these rare
tumors, we reviewed the clinicopathologic and immunohistochemical findings of four cases of primary esophageal
carcinoid. The age of the patients ranged from 48 to 82 years (mean 63 years; median 61 years). The lower segment of the esophagus was involved in two cases and the mid segment was involved in one case. The sizes of the
tumors ranged from 0.3 cm to 3.5 cm. Two
tumors were confined to the lamina propria and two invaded into the muscular wall. Two
tumors appeared polypoid, whereas the remaining two were incidental findings and associated with
adenocarcinoma arising in a background of
Barrett esophagus. The
adenocarcinoma was superficially invasive in one case, whereas it penetrated the muscular wall in the other. All four
carcinoid tumors were immunoreactive with
chromogranin and
synaptophysin. There was focal expression of
serotonin in two cases,
glucagon in one case, and
pancreatic polypeptide in one case. Endocrine cell
hyperplasia was noted in both the
Barrett esophagus and the invasive
adenocarcinoma. One patient died secondary to postoperative
pneumonia. Three patients are alive and disease free at 1, 6, and 23 years status post
therapy. None of the patients had metastatic disease. These findings show that esophageal
carcinoids are associated with a favorable prognosis. They arise in two settings: (1) a single large polypoid
tumor or (2) an incidental finding and in association with
adenocarcinoma arising in the background of
Barrett esophagus. The presence of endocrine cell
hyperplasia in the Barrett mucosa and the
adenocarcinoma supports the hypothesis that these lesions arise from a common stem cell.