2021 Volume 35 Issue 2 Pages 160-167
A 50s-year-old man had jaundice during regular consultation for primary macroglobulinemia. Early phase in abdominal contrast-enhanced CT scan detected 19mm mass with weakly enhancement effect at intrapancreatic bile duct with dilated central bile duct. EUS revealed the hypoechoic mass with 19mm diameter at the terminal of bile duct. Biopsy taken from the tumor under ERC revealed the possibility of neuroendocrine carcinoma (NEC) or mixed neuroendocrine non-neuroendocrine neoplasm (MiNEN). The patient underwent pylorus resected pancreaticoduodenectomy. Histopathological examination of resected specimen showed the tumor consisted of two components, NEC and adenocarcinoma. The proportions of NEC and adenocarcinoma components were 70 and 30%, respectively. Finally, histopathological diagnosis was MiNEN of extra hepatic bile duct. We report a rare case of MiNEN of extra hepatic bile duct and review the literatures regarding clinicopathological features and adjuvant chemotherapy for MiNEN.