Background : Neuroendocrine carcinoma originating from extra hepatic bile duct is very rare and only a few cases have been reported. Because of its scarcity of incidence, not much is known about the disease but for its aggressiveness and poor prognosis. Herein we report two cases of large cell neuroendocrine carcinoma (LCNEC) originating from extrahepatic bile duct.

Methods : Case 1. A 60-years-old woman visited the hospital with jaundice. She was preoperatively diagnosed a perihilar cholangiocarcinoma, and a left hepatectomy and caudectomy with hepaticojejunostomy was performed. From the histopathological findings, we diagnosed the tumor as a LCNEC (pT2aN1M0, pStage IIIB) with focal proportion of an adenocarcinoma component. The postoperative course was uneventful, and she was administered etoposide and cisplatin every 3 weeks (6th cycles) as an adjuvant chemotherapy. At the 6-month follow-up, liver metastases were detected, and despite multiple cycles of chemotherapy with various regimens, the patient expired 24 months post-operation.

Results : Case 2. A 67-years-old man visited the hospital with jaundice. He was diagnosed a cholangiocarcinoma of mid- common bile duct (CBD) and underwent laparoscopic pylorus-preserving pancreatoduodenectomy (PPPD). The pathological findings showed a LCNEC (pT1N1M0, pStage IIB) with focal proportion of an adenocarcinoma component in the extrahepatic bile duct with lymph node metastases. After recovery, he was administered etoposide and cisplatin every 3 weeks (6th cycle) as an adjuvant chemotherapy. Liver metastasis was detected 23 months after surgery, leading to multiple cycles of chemotherapy with various regimens and radiotherapy. Despite multiple metastasis, he is still alive 36 months after surgery.

Conclusions : Since LCNEC is very rare and has a poor prognosis, more research is needed to establish a definite treatment approach.