Esophageal endocrinomas, an extremely rare tumor: a statistical comparative evaluation of 28 ordinary carcinoids and 72 atypical variants.

Esophageal endocrinomas are an extremely rare neoplasm. The present study aimed to evaluate multiphasic clinical and pathologic aspects of 100 cases of esophageal endocrinomas, consisting of 28 cases of ordinary type carcinoids and 72 atypical varieties collected from worldwide literature and recorded on computer in the Niigata Registry. The evaluation of esophageal endocrinoma cases was on most occasions carried out in accordance with a comparison between ordinary carcinoids and atypical varieties. Esophageal endocrinomas were generally characterized by a preponderance of male patients and an older age group. They also exhibited a higher metastasis rate, and a lower incidence of carcinoid syndrome than overall endocrinomas of the digestive series. They further indicated a probable unfavorable postoperative outcome as compared to endocrinomas in most other digestive organs. Ordinary carcinoids of the esophagus were characterized by smaller size at detection, lower metastasis rate, and better postoperative outcome than the atypical variety group. Because of the extremely small number of cases of esophageal endocrinomas, additional case reports are in future expected to be satisfactorily documented as a valuable record.