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Intraductal Papillary Mucinous Neoplasm (IPMN) with Extra-Pancreatic Mucin: A Case Series and Review of the Literature

  • Original Article
  • Published:
Journal of Gastrointestinal Surgery

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.

Aim

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.

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Authors and Affiliations

  1. Department of Surgery, University of Virginia Health System, Medical Research Building #4, Room 3116, P.O. Box 801359, Charlottesville, VA, 22908, USA

    Laura H. Rosenberger

  2. Department of Surgery, Yale–New Haven Hospital, New Haven, CT, USA

    Louis H. Stein

  3. Department of Pathology, Thomas Jefferson University, Philadelphia, PA, USA

    Agnieszka K. Witkiewicz

  4. Department of Surgery, and the Jefferson Pancreas, Biliary and Related Cancer Center, Thomas Jefferson University, Philadelphia, PA, USA

    Eugene P. Kennedy & Charles J. Yeo

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  1. Laura H. Rosenberger
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  2. Louis H. Stein
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Correspondence to Laura H. Rosenberger.

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Rosenberger, L.H., Stein, L.H., Witkiewicz, A.K. et al. Intraductal Papillary Mucinous Neoplasm (IPMN) with Extra-Pancreatic Mucin: A Case Series and Review of the Literature. J Gastrointest Surg 16, 762–770 (2012). https://doi.org/10.1007/s11605-012-1823-8

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