title: Intraductal papillary mucinous neoplasm of the biliary tract: a real disease?
abstract: BACKGROUND
  Despite increasing numbers of reports, biliary tract intraductal papillary mucinous neoplasm (BT-IPMN) is not yet recognized as a unique neoplasm. The aim of the present study was to define the presence of BT-IPMN in a large series of resected biliary neoplasms.
  
  
  METHODS
  From May 1994 to December 2006, BT-IPMN cases were identified by reviewing pathology specimens of all resected cholangiocarcinomas and other biliary neoplasms when cystic, papillary or mucinous features were cited in pathology reports.
  
  
  RESULTS
  BT-IPMN was identified in 23 out of 253 (9%) specimens using the strict histopathological criteria of IPMN. The most common presenting symptom was abdominal discomfort which was present in 15 patients (65%). Only one of the original operative pathology reports used the term IPMN; 16 (70%) used the terms cystic, mucinous and/or papillary. BT-IPMN was isolated to non-hilar extra-hepatic ducts in 12 (52%), intra-hepatic ducts in 6 (26%) and hilar extra-hepatic ducts in 5 patients (22%). Carcinoma was found in association with BT-IPMN in 19 patients (83%); 5-year survival was 38% after resection.
  
  
  CONCLUSIONS
  BT-IPMN occurs throughout the intra- and extra-hepatic biliary system and can be identified readily as a unique neoplasm. Broader acceptance of BT-IPMN as a unique neoplasm may lead to a better understanding of the pathogenesis of biliary malignancies.
authors.forename: Joshua G
authors.initials: JG
authors.lastname: Barton
authors.forename: David A
authors.initials: DA
authors.lastname: Barrett
authors.forename: Marco A
authors.initials: MA
authors.lastname: Maricevich
authors.forename: Thomas
authors.initials: T
authors.lastname: Schnelldorfer
authors.forename: Christina M
authors.initials: CM
authors.lastname: Wood
authors.forename: Thomas C
authors.initials: TC
authors.lastname: Smyrk
authors.forename: Todd H
authors.initials: TH
authors.lastname: Baron
authors.forename: Michael G
authors.initials: MG
authors.lastname: Sarr
authors.forename: John H
authors.initials: JH
authors.lastname: Donohue
authors.forename: Michael B
authors.initials: MB
authors.lastname: Farnell
authors.forename: Michael L
authors.initials: ML
authors.lastname: Kendrick
authors.forename: David M
authors.initials: DM
authors.lastname: Nagorney
authors.forename: Kaye M
authors.initials: KM
authors.lastname: Reid Lombardo
authors.forename: Florencia G
authors.initials: FG
authors.lastname: Que