A Case of Liver Metastasis of Primary Neuroendocrine Carcinoma of the Bile Duct Treated with Chemotherapy

Abstract

A 78-year-old man was admitted to our hospital for investigation of liver function abnormalities. Contrast-enhanced computed tomography (CT) showed a high-enhanced tumor in the lower bile duct. Endoscopic retrograde cholangiopancreatography showed stenosis of the lower bile duct and dilation of the upper bile duct. Biopsy of the tumor suggested small cell neuroendocrine carcinoma, and subtotal stomach-preserving pancreatoduodenectomy was performed. Abdominal CT performed 12 months after the surgery showed two metastases in the liver. Chemotherapy was started with etoposide (ETP) and carboplatin (CBDCA). After the chemotherapy, the liver metastases shrank. The patient has remained alive more than 20 months after surgery. Primary NEC of the bile duct is extremely rare, and its prognosis is reported to be extremely poor. The chemotherapy was effective in the present case. Therefore, this case is reported along with a short discussion of the literature.