title: Clinicopathological characteristics of biliary neuroendocrine neoplasms: a multicenter study.
abstract: OBJECTIVE
  This study assessed the clinicopathological features, therapeutic approaches, and prognosis of patients with biliary neuroendocrine neoplasm (NENs).
  
  
  METHODS
  Multicenter retrospective study of patients with biliary tract NENs in the gallbladder, the extrahepatic bile duct, or the ampulla of Vater between 2005 and 2014.
  
  
  RESULTS
  Total of 43 patients were included in the study. The median age was 62 years (range: 29-84 years) and 58.1% of the patients were male. The tumors occurred in the gallbladder (n = 11), the extrahepatic bile duct (n = 5) or the ampulla of Vater (n = 27). The liver was the most common metastatic site. Based on the 2010 World Health Organization classification, more patients with gallbladder NENs (11/11 (100%)) had neuroendocrine carcinoma G3 than those with NENs in the ampulla of Vater (10/27 (37.1%)). The median progression free survival time (39.3 vs 5.1 months, p = 0.001) and median overall survival time (46.9 vs 7.9 months, p < 0.001) were significantly longer in patients with ampulla of Vater NENs than gallbladder NENs. A 2010 World Health Organization classification of neuroendocrine carcinoma G3 was independently related to poor overall survival (hazard ratio (HR), 27.1; 95% confidence intervals (CI), 2.81-260.68; p = 0.004).
  
  
  CONCLUSIONS
  The 2010 World Health Organization classification of neuroendocrine carcinoma G3 was the only factor related to poor prognosis in patients with biliary neuroendocrine neoplasms.
authors.affiliation: a Department of Internal Medicine , Yonsei University Wonju College of Medicine , Wonju , South Korea.
authors.forename: Kyong Joo
authors.initials: KJ
authors.lastname: Lee
authors.affiliation: b Department of Internal Medicine , Gachon university, Gil Medical Center , Incheon , South Korea.
authors.forename: Jae Hee
authors.initials: JH
authors.lastname: Cho
authors.affiliation: c Department of Internal Medicine and Liver Research Institute , Seoul National University College of Medicine , Seoul , South Korea.
authors.forename: Sang Hyub
authors.initials: SH
authors.lastname: Lee
authors.affiliation: d Department of Internal Medicine , Samsung Medical Center, Sungkyunkwan University School of Medicine , Seoul , South Korea.
authors.forename: Kwang Hyuk
authors.initials: KH
authors.lastname: Lee
authors.affiliation: e National Health Insurance Corporation Ilsan Hospital , Goyang , South Korea.
authors.forename: Byung Kyu
authors.initials: BK
authors.lastname: Park
authors.affiliation: f Department of Internal Medicine , Dongguk University Ilsan Hospital, Dongguk University , Goyang , South Korea.
authors.forename: Jun Kyu
authors.initials: JK
authors.lastname: Lee
authors.affiliation: g Center for Liver Cancer, National Cancer Center , Goyang , South Korea.
authors.forename: Sang Myung
authors.initials: SM
authors.lastname: Woo
authors.affiliation: c Department of Internal Medicine and Liver Research Institute , Seoul National University College of Medicine , Seoul , South Korea.
authors.forename: Ji Kon
authors.initials: JK
authors.lastname: Ryu
authors.affiliation: d Department of Internal Medicine , Samsung Medical Center, Sungkyunkwan University School of Medicine , Seoul , South Korea.
authors.forename: Jong Kyun
authors.initials: JK
authors.lastname: Lee
authors.affiliation: b Department of Internal Medicine , Gachon university, Gil Medical Center , Incheon , South Korea.
authors.forename: Yeon Suk
authors.initials: YS
authors.lastname: Kim
authors.affiliation: a Department of Internal Medicine , Yonsei University Wonju College of Medicine , Wonju , South Korea.
authors.forename: Jae Woo
authors.initials: JW
authors.lastname: Kim
authors.affiliation: g Center for Liver Cancer, National Cancer Center , Goyang , South Korea.
authors.forename: Woo Jin
authors.initials: WJ
authors.lastname: Lee
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001661:Biliary Tract Neoplasms / Q000145:classification* / Q000473:pathology* / Q000628:therapy; D018278:Carcinoma, Neuroendocrine / Q000145:classification* / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D056910:Republic of Korea; D012189:Retrospective Studies; D016019:Survival Analysis
keywords: World Health Organization classification; biliary tract; neuroendocrine carcinoma; neuroendocrine neoplasm; prognosis