title: Intraductal papillary mucinous neoplasm (IPMN) with extra-pancreatic mucin: a case series and review of the literature.
abstract: BACKGROUND
  Intraductal papillary mucinous neoplasm (IPMN) is an increasingly recognized pancreatic neoplasm characterized by excessive mucin secretion by ductal epithelial cells resulting in a cystic dilation of the pancreatic duct.
  
  
  OBJECTIVE
  The objective of this study was to review Thomas Jefferson University's experience and the literature to determine the significance of extra-pancreatic mucin when associated with an IPMN.
  
  
  RESULTS
  A retrospective analysis at our institution revealed only two cases of IPMN associated with extra-pancreatic mucin, which were classic IPMNs with rupture of the pancreatic duct and peritoneal mucin spillage. This specific finding is not previously described, although is assumed as five cases were reported in the literature with IPMN and mucin extension demonstrated by pseudomyxoma peritonei (PMP). We propose IPMN of the pancreas may be grossly compared to a mucocele of the appendix, as both are characterized by excessive secretion of mucin by ductal epithelial cells. A morbid complication of a mucocele is PMP. The presence of extra-pancreatic mucin with an IPMN could present a rare but important marker of the eventual seeding of tumor outside the primary IPMN. This has been documented with cases of iatrogenic spilling of pancreatic mucin, as well as multiple cases of IPMN associated with pseudomyxoma peritonei.
  
  
  CONCLUSIONS
  At this time, there is scant reporting and consensus for the treatment of IPMN with extra-pancreatic mucin.
authors.affiliation: Department of Surgery, University of Virginia Health System, Medical Research Building #4, P.O. Box 801359, Charlottesville, VA 22908, USA. LHR2M@virginia.edu
authors.forename: Laura H
authors.initials: LH
authors.lastname: Rosenberger
authors.forename: Louis H
authors.initials: LH
authors.lastname: Stein
authors.forename: Agnieszka K
authors.initials: AK
authors.lastname: Witkiewicz
authors.forename: Eugene P
authors.initials: EP
authors.lastname: Kennedy
authors.forename: Charles J
authors.initials: CJ
authors.lastname: Yeo
mesh_terms: D000368:Aged; D006801:Humans; D008297:Male; D009077:Mucins / Q000378:metabolism*; D009366:Neoplasm Seeding; D018297:Neoplasms, Cystic, Mucinous, and Serous / Q000150:complications / Q000378:metabolism* / Q000601:surgery; D010183:Pancreatic Ducts; D010190:Pancreatic Neoplasms / Q000150:complications / Q000378:metabolism* / Q000601:surgery; D010534:Peritoneal Neoplasms / Q000209:etiology* / Q000628:therapy; D011553:Pseudomyxoma Peritonei / Q000209:etiology / Q000628:therapy; D012189:Retrospective Studies; D012422:Rupture, Spontaneous / Q000209:etiology
chemical_list: D009077:Mucins