Intraductal papillary mucinous neoplasm of the biliary tract: A real disease?

Joshua G. Barton, David A. Barrett, Marco A. Maricevich, Thomas Schnelldorfer, Christina M. Wood, Thomas C. Smyrk, Todd H. Baron, Michael G. Sarr, John H. Donohue, Michael B. Farnell, Michael L. Kendrick, David M. Nagorney, Kaye M.Reid Lombardo, Florencia G. Que

Research output: Contribution to journalArticle

53 Scopus citations


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. Conclusion: 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.

Original languageEnglish (US)
Pages (from-to)684-691
Number of pages8
Issue number8
StatePublished - 2009


  • Biliary
  • Intraductal papillary mucinous neoplasm
  • Mucinous
  • Papillary

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

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    Barton, J. G., Barrett, D. A., Maricevich, M. A., Schnelldorfer, T., Wood, C. M., Smyrk, T. C., Baron, T. H., Sarr, M. G., Donohue, J. H., Farnell, M. B., Kendrick, M. L., Nagorney, D. M., Lombardo, K. M. R., & Que, F. G. (2009). Intraductal papillary mucinous neoplasm of the biliary tract: A real disease? HPB, 11(8), 684-691.