De Novo Esophageal Small-Cell Neuroendocrine Carcinoma in a Patient With Achalasia: A Case Report

Esophageal neuroendocrine carcinoma (NEC) is rare and aggressive, representing 0.4% to 2% of esophageal cancers, with small-cell NEC comprising 95% of cases. Achalasia is known to increase the risk for esophageal malignancy, particularly squamous cell carcinoma. We describe an 81-year-old woman with longstanding achalasia who developed dysphagia. Endoscopy showed a fungating mass, and biopsy confirmed small-cell NEC. She was treated with platinum–etoposide chemotherapy, resulting in complete resolution. This case demonstrates remission with chemotherapy alone, challenging traditional surgical approaches. To our knowledge, this is the first reported case of esophageal small-cell NEC arising in conjunction with achalasia. Further research is warranted to establish optimal management strategies for such rare presentations.