A large cell neuroendocrine carcinoma of the esophagus

Neuroendocrine carcinomas generally have a poor prognosis, and large-cell types are rare. We report a case of large cell neuroendocrine carcinoma of the esophagus with long-term survival after surgical treatment. A 56-year-old man, previously hospitalized for dysphasia, was referred to our hospital with a diagnosis of neuroendocrine carcinoma of the esophagus. A localized polypoid tumor was detected in the middle thoracic esophagus, and on the basis of a diagnosis of T1bN0M0, Stage I neuroendocrine carcinoma, thoracoscopic esophagectomy was performed. Histopathological and immunohistochemical findings were cytokeratin AE1/AE3-positive, chromogranin A-positive, synaptophysin-positive, CD56-positive, p63-negative, Ki-67 (labeling index) 81.8%, and many mitotic figures. The final diagnosis was large cell neuroendocrine carcinoma, categorized as pT1b (SM1), ly1, v0, pN0, pStage I. The patient has been free from recurrence for 3 years and 5 months after surgery without any adjuvant therapy. We consider that this case of long-term survival after surgery alone was because the carcinoma was superficial without any lymph node metastasis.