2019 Volume 80 Issue 8 Pages 1525-1530
A 62-year-old woman was admitted for epigastric pain and jaundice. Abdominal enhanced computed tomography (CT) showed a high-density tumor in the lower bile duct and a dilated common bile duct. After biopsy, we suspected adenocarcinoma in the lower bile duct and performed pancreatoduodenectomy. Tumor cells with a high nuclear-cytoplasmic (N/C) ratio and nested pattern were confirmed by histological examination. Immunostaining findings were positive for chromogranin A and synaptophysin. The Ki-67 index was > 90%. Consequently, we diagnosed large cell neuroendocrine carcinoma (LCNEC) in the lower bile duct. As adjuvant chemotherapy, cisplatin and irinotecan were administered. However, three months after surgery, a recurrence was observed in the lymph nodes. Ten months after surgery, the patient died due to recurrence in the lymph nodes, multiple liver metastases, and peritoneal dissemination. LCNEC in the bile duct is extremely rare and appears to be highly aggressive with early recurrence and metastases.