An Uncommon Encounter: A Comprehensive Case Report of an Advanced Esophageal Neuroendocrine Carcinoma

Esophageal neuroendocrine carcinomas (E-NECs) are rare malignant tumors with unknown etiology and pathogenesis. The aggressive nature of E-NECs coupled with a tendency to metastasize and no available treatment guidelines lead to poor prognosis. Here, we report a case of a 65-year-old, previously healthy female who presented with difficulty in swallowing solids, burning sensation over the epigastric region, weight loss (>10%), and altered bowel habits for the last three months. Contrast-enhanced CT (CECT) thorax revealed asymmetric mid-esophageal wall thickening with lymphadenopathy and metastasis in both hepatic lobes. Esophageal endoscopy revealed a large circumferential ulcero-proliferative mass and umbilicated lesions. A histopathological examination revealed small cells with scant cytoplasm and pleomorphic, hyperchromatic nuclei with prominent nuclear molding, and extensive necrosis. Immunohistochemistry revealed positivity for synaptophysin, chromogranin A, and CD56. Ki-67 index was 53%. These findings suggested poorly differentiated, small cell type, high-grade E-NEC. Chemotherapy with cisplatin (30mg/m²) + irinotecan (60mg/m²) was initiated. However, following two chemotherapy cycles, the patient succumbed.