Published online Aug 14, 2016. doi: 10.3748/wjg.v22.i30.6960
Peer-review started: March 31, 2016
First decision: May 12, 2016
Revised: May 24, 2016
Accepted: June 13, 2016
Article in press: June 13, 2016
Published online: August 14, 2016
Processing time: 129 Days and 7.5 Hours
Neuroendocrine carcinoma (NEC) originating from the gastrointestinal hepatobiliary-pancreas is a rare, invasive, and progressive disease, for which the prognosis is extremely poor. The patient was a 72-year-old man referred with complaints of jaundice. He was diagnosed with middle extrahepatic cholangiocarcinoma (cT4N1M0, cStage IV). He underwent a right hepatectomy combined with extrahepatic bile duct and portal vein resection after percutaneous transhepatic portal vein embolization. Microscopic examination showed a large-cell neuroendocrine carcinoma according to the WHO criteria for the clinicopathologic classification of gastroenteropancreatic neuroendocrine tumors. Currently, the patient is receiving combination chemotherapy with cisplatin and etoposide for postoperative multiple liver metastases. Although NEC is difficult to diagnose preoperatively, it should be considered an uncommon alternative diagnosis.
Core tip: We encountered a resected case of a neuroendocrine carcinoma originating from the extrahepatic bile duct. Although neuroendocrine carcinoma is rare and difficult to diagnose preoperatively, it should be considered an uncommon alternative diagnosis.