title: A case report of primary neuroendocrine carcinoma of the perihilar bile duct.
abstract: BACKGROUND
  Although neuroendocrine tumors are most commonly found in the digestive system, neuroendocrine tumors originating from the bile duct are rare, and neuroendocrine carcinomas derived from the perihilar bile duct are extremely rare. This report presents the clinical course and clinicopathological features of neuroendocrine carcinomas arising from the extrahepatic bile duct.
  
  
  METHODS
  A 70-year-old Japanese woman was preoperatively diagnosed with perihilar cholangiocarcinoma, and a radical resection with an extended left hepatic lobectomy and a choledochojejunostomy was performed. From the histopathological findings, we diagnosed the tumor as a neuroendocrine carcinoma of the bile duct (small cell type) with lymph node metastasis. The patient was treated with the same adjuvant chemotherapy as that used for small cell carcinoma of the lung. At 10 months after surgery, there was no recurrence of the disease.
  
  
  CONCLUSIONS
  Neuroendocrine carcinoma of the extrahepatic biliary tracts is a very rare and highly malignant disease with a poor prognosis. A multidisciplinary approach could improve the prognosis for this neoplasm.
authors.affiliation: Division of General Surgery, Japanese Red Cross Kumamoto Hospital, Nagamineminami 2-1-1, Higashiku, Kumamoto city, Kumamoto, 861-8520, Japan. flinge21@yahoo.co.jp.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kihara
authors.affiliation: Division of General Surgery, Japanese Red Cross Kumamoto Hospital, Nagamineminami 2-1-1, Higashiku, Kumamoto city, Kumamoto, 861-8520, Japan. h-yokomizo@kumamoto-med.jrc.or.jp.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yokomizo
authors.affiliation: Division of Gastroenterology, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan. tagatame@yahoo.co.jp.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Urata
authors.affiliation: Division of Diagnostic Pathology, Japanese Red Cross Kumamoto Hospital, Kumamoto, Japan. nagamine.michiko.jx@kumamoto-med.jrc.or.jp.
authors.forename: Michiko
authors.initials: M
authors.lastname: Nagamine
authors.affiliation: Division of General Surgery, Japanese Red Cross Kumamoto Hospital, Nagamineminami 2-1-1, Higashiku, Kumamoto city, Kumamoto, 861-8520, Japan. krchirata@kumamoto-med.jrc.or.jp.
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Hirata
mesh_terms: D000368:Aged; D001650:Bile Duct Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D017734:Bile Ducts, Extrahepatic / Q000473:pathology* / Q000601:surgery; D018278:Carcinoma, Neuroendocrine / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D003937:Diagnosis, Differential; D005260:Female; D006498:Hepatectomy; D006801:Humans; D018285:Klatskin Tumor / Q000175:diagnosis; D008207:Lymphatic Metastasis; D011379:Prognosis