Large cell neuroendocrine carcinoma of the esophagus: report of a rare case and a literature review

Neuroendocrine carcinoma of the esophagus, mostly comprised of the small cell type, is a rare malignant neoplasm and accounts for only 3.3% of all esophageal malignant tumors. We present a rare case of a large cell neuroendocrine carcinoma in a 73-year-old man with dysphagia. Chest computed tomography showed a 5-cm eccentric thickening of the upper thoracic esophageal wall and enlarged paratracheal lymph nodes. A McKeown operation was performed, and the resected tumor consisted of solid nests of large cells with round hyperchromatic nuclei, conspicuous nucleoli, and abundant eosinophilic cytoplasm. Immunopositivity of the tumor cells for synaptophysin and chromogranin confirmed their neuroendocrine nature. A squamous cell carcinoma or adenocarcinoma component was absent. Our report is the first description in South Korea of a tumor with typical clinical and histological features of a pure large cell neuroendocrine carcinoma of the esophagus.