A Case of Esophageal Neuroendocrine Carcinoma for Which Irinotecan and Cisplatin Combination Therapy Was Effective

Abstract A 72-year-old previously healthy man visited our hospital with complaints of hoarseness and dysphagia. Computed tomography showed wall thickening of the thoracic esophagus; invasion to the left main bronchus, aorta, and right supraclavicular lymph nodes (LNs); right recurrent nerve LNs; and cardiac LN swelling. Esophagogastroduodenoscopy revealed an elevated tumor in the middle thoracic esophagus, which was similar to a submucosal tumor and had a longitudinal ulcer at its center. Pathologicexamination showed a tumor with a high N/C ratio, and immunohistochemical staining showed the tumor was CD56 and NSE positive, with a Ki-67 index >80%. We diagnosed esophageal neuroendocrine carcinoma (NEC), cT4N3M0, Stage IVa. We started chemotherapy with irinotecan and cisplatin (IP therapy) according to a regimen for small-cell lung cancer. After 3 courses of chemotherapy, the primary lesion and the LN swelling had almost disappeared. Esophageal NEC is relatively rare disease, so there are no standard established treatments. We report a case of esophageal NEC for which IP therapy was effective with the relevant literature cited.