Long-term survival with multimodal treatment including conversion surgery for locally advanced esophageal neuroendocrine carcinoma: A case report

BACKGROUND: Esophageal neuroendocrine carcinoma (NEC), a rare and aggressive malignancy with a poor prognosis, is often diagnosed at an advanced stage. The optimal treatment strategy for locally advanced and recurrent esophageal NEC remains unclear, and conversion surgery has only been reported for a few cases. Herein, we present the case of a 66-year-old male with locally advanced esophageal NEC initially diagnosed as squamous cell carcinoma. CASE SUMMARY: The patient underwent induction chemotherapy with docetaxel, cisplatin, and 5-fluorouracil, followed by conversion surgery, including subtotal esophagectomy, three-field lymph node dissection, and distal pancreatectomy with splenectomy, due to infiltration of the pancreas by the No. 11p lymph node. Postoperative pathological findings revealed a large cell-type NEC without a squamous cell carcinoma component, suspected to be a mixed neuroendocrine/non-neuroendocrine neoplasm. Hepatic metastasis was diagnosed within one month of surgery. Despite the administration of four courses of irinotecan + cisplatin chemotherapy, the treatment effect was considered a 'progressive disease'. After a multidisciplinary discussion, the patient underwent partial liver resection, followed by second-line chemotherapy with amrubicin. The patient achieved three-year survival with no new recurrence. CONCLUSION: This case highlights the potential of multimodal treatment for long-term survival in advanced esophageal NEC.