Neuroendocrine carcinoma of the esophagus: Report of a case and review of the literature

Abstract

Esophageal neuroendocrine carcinomas are rare, aggressive and have a poor prognosis. Combined therapy using chemotherapy, radiotherapy and/or surgery appear effective for them. Upper gastrointestinal endoscope of a 65-year-old male revealed a localized ulcerative lesion in the middle esophagus. Histology of biopsy specimens indicated a neuroendocrine carcinoma. A computed tomography showed an esophageal tumor with enlarged mediastinal lymph nodes. The patient was administered neoadjuvant chemo-therapy consisting of 5-fluorouracil and cisplatin, which led to partial response. Subtotal esophagectomy with three-field lymphadenectomy was performed. Pathologically, the tumor was 25 mm and infiltrated the proper muscle layer. The tumor cells were arranged in microtubular structures, with small and round cells containing scanty cytoplasm, and exhibited intense mitosis 51/10 HPF. They were positive for synaptophysin and chromogranin A, and Ki-67 labeling index was 70% - 80%. These findings led to the diagnosis of neuroendocrine carcinoma of small cell type. The patient was administered adjuvant chemotherapy using cisplatin and CPT-11, and he is now alive disease-free at the time of this writing.