title: Clinical features of 20 patients with curatively resected biliary neuroendocrine tumours.
abstract: BACKGROUND
  Neuroendocrine tumours very rarely occur in the biliary tract; information about them is limited.
  
  
  OBJECTIVE
  To present the clinical characteristics and prognosis of curatively resected biliary neuroendocrine tumours.
  
  
  METHODS
  Review of medical records dated between 2000 and 2010 of 20 patients from three medical centres with biliary neuroendocrine tumour based on curative resection.
  
  
  RESULTS
  Based on the World Health Organization 2010 classification, five and one patients had neuroendocrine tumour grades 1 and 2, seven had neuroendocrine carcinoma, and seven were diagnosed with mixed adenoneuroendocrine carcinoma. The locations were the following: seven in the gallbladder, four in the extrahepatic bile duct, and nine in the ampulla of Vater. Lymph node and hepatic metastases were noted in 11 and 4 patients, respectively. Fourteen patients experienced recurrence; most had recurrence in the liver. Patients with neuroendocrine tumour grade 1 had a lower rate of recurrence compared to others (p=0.001). The median disease-free and overall survival times were 5.8 (0.4-53.6) and 13.7 (1.9-102.1) months for all four subtypes. However, the median disease free and overall survival rates of neuroendocrine tumours were significantly longer than those of neuroendocrine carcinomas or mixed adenoneuroendocrine carcinoma.
  
  
  CONCLUSIONS
  Patients with biliary neuroendocrine tumour showed extremely different clinical outcomes according to histopathologic subtypes by World Health Organization 2010 classification.
authors.affiliation: Department of Internal Medicine and Liver Research Institute, Seoul National University College of Medicine, Seoul, South Korea.
authors.forename: Jaihwan
authors.initials: J
authors.lastname: Kim
authors.forename: Woo Jin
authors.initials: WJ
authors.lastname: Lee
authors.forename: Sang Hyub
authors.initials: SH
authors.lastname: Lee
authors.forename: Kyoung Bun
authors.initials: KB
authors.lastname: Lee
authors.forename: Ji Kon
authors.initials: JK
authors.lastname: Ryu
authors.forename: Yong-Tae
authors.initials: YT
authors.lastname: Kim
authors.forename: Sun-Whe
authors.initials: SW
authors.lastname: Kim
authors.forename: Yong Bum
authors.initials: YB
authors.lastname: Yoon
authors.forename: Jin Hyeok
authors.initials: JH
authors.lastname: Hwang
authors.forename: Ho-Seong
authors.initials: HS
authors.lastname: Han
authors.forename: Sang Myung
authors.initials: SM
authors.lastname: Woo
authors.forename: Sang Jae
authors.initials: SJ
authors.lastname: Park
mesh_terms: D000328:Adult; D000368:Aged; D014670:Ampulla of Vater / Q000473:pathology* / Q000601:surgery; D018278:Carcinoma, Neuroendocrine / Q000473:pathology / Q000556:secondary / Q000601:surgery; D003138:Common Bile Duct Neoplasms / Q000473:pathology* / Q000601:surgery; D018572:Disease-Free Survival; D005260:Female; D005706:Gallbladder Neoplasms / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary*; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000473:pathology* / Q000556:secondary / Q000601:surgery; D012189:Retrospective Studies