Current Gastroenterology Reports

, Volume 5, Issue 2, pp 133–140

Intraductal papillary mucinous tumors of the pancreas

  • Friedrich H. Schmitz-Winnenthal
  • Kaspar Z’graggen
  • Christine Volk
  • Bruno M. Schmied
  • Markus W. Büchler


Cystic neoplasms of the exocrine pancreas are a small fraction of pancreatic tumors. Within that group of cystic neoplasms, intraductal papillary mucinous tumors (IPMTs) can be distinguished from mucinous cystic neoplasms, serous cystic neoplasms, and pseudopapillary cystic tumors. Awareness of IPMTs has increased since the World Health Organization classified these tumors as its own group in 1996. Because of their favorable prognosis, an extensive diagnostic workup for IPMTs should be performed in patients presenting with cystic lesions of the pancreas. This workup often leads to the diagnosis and the predominant tumor location and size, although the extent of the ductal changes can only be established by histopathology. Surgical resection is the therapy of choice for IPMTs. The type of resection depends upon the extent of the quantitative and qualitative ductal involvement. Total pancreatectomy is currently the treatment for an IPMT that comprises the entire main duct.


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References and Recommended Reading

  1. 1.
    Moesinger RC, Talamini MA, Hruban RH, et al.: Large cystic pancreatic neoplasms: pathology, resectability, and outcome. Ann Surg Oncol 1999, 6:682–690.PubMedCrossRefGoogle Scholar
  2. 2.
    Adsay NV, Conlon KC, Zee SY, et al.: Intraductal papillary-mucinous neoplasms of the pancreas: an analysis of in situ and invasive carcinomas in 28 patients. Cancer 2002, 94:62–77. This paper demonstrates that in situ and invasive carcinoma may be more common than previously recognized in IPMTs. The neoplasms are less aggressive as a group than conventional pancreatic ductal adenocarcinoma, but IPMTs may be fatal, even in the absence of identifiable invasive carcinoma.PubMedCrossRefGoogle Scholar
  3. 3.
    Warshaw AL: Mucinous cystic tumors and mucinous ductal ectasia of the pancreas. Gastrointest Endosc 1991, 37:199–201.PubMedGoogle Scholar
  4. 4.
    Bastid C, Bernard JP, Sarles H, et al.: Mucinous ductal ectasia of the pancreas: a premalignant disease and a cause of obstructive pancreatitis. Pancreas 1991, 6:15–22.PubMedCrossRefGoogle Scholar
  5. 5.
    Warshaw AL, Berry J, Gang DL: Villous adenoma of the duct of Wirsung. Dig Dis Sci 1987, 32:1311–1313.PubMedCrossRefGoogle Scholar
  6. 6.
    Kohler B, Kohler G, Riemann JF: Mucinous cystadenoma of the pancreas: exact endoscopic assessment with pancreoscopy. Leber Magen Darm 1990, 20:196–199.PubMedGoogle Scholar
  7. 7.
    Nickl NJ, Lawson JM, Cotton PB: Mucinous pancreatic tumors: ERCP findings. Gastrointest Endosc 1991, 37:133–138.PubMedGoogle Scholar
  8. 8.
    Morohoshi T, Kanda M, Asanuma K, Kloppel G: Intraductal papillary neoplasms of the pancreas: a clinicopathologic study of six patients. Cancer 1989, 64:1329–1335.PubMedCrossRefGoogle Scholar
  9. 9.
    Itai Y, Ohhashi K, Nagai H, et al.: ’Ductectatic’ mucinous cystadenoma and cystadenocarcinoma of the pancreas. Radiology 1986, 161:697–700.PubMedGoogle Scholar
  10. 10.
    Itai Y, Kokubo T, Atomi Y, et al.: Mucin-hypersecreting carcinoma of the pancreas. Radiology 1987, 165:51–55.PubMedGoogle Scholar
  11. 11.
    Conley CR, Scheithauer BW, van Heerden JA, Weiland LH: Diffuse intraductal papillary adenocarcinoma of the pancreas. Ann Surg 1987, 205:246–249.PubMedCrossRefGoogle Scholar
  12. 12.
    Stommer P, Gebhardt C, Schultheiss KH: Adenocarcinoma of the pancreas with a predominant intraductal component: a special variety of ductal adenocarcinoma. Pancreas 1990, 5:114–118.PubMedCrossRefGoogle Scholar
  13. 13.
    Gigot JF, Deprez P, Sempoux C, et al.: Surgical management of intraductal papillary mucinous tumors of the pancreas: the role of routine frozen section of the surgical margin, intraoperative endoscopic staged biopsies of the Wirsung duct, and pancreaticogastric anastomosis. Arch Surg 2001, 136:1256–1262.PubMedCrossRefGoogle Scholar
  14. 14.
    Fukushima N, Mukai K, Sakamoto M, et al.: Invasive carcinoma derived from intraductal papillary-mucinous carcinoma of the pancreas: clinicopathologic and immunohistochemical study of eight cases. Virchows Arch 2001, 439:6–13.PubMedCrossRefGoogle Scholar
  15. 15.
    Yamaguchi K, Tanaka M: Atlas of Cystic Neoplasms of the Pancreas. Tokyo; Basel: Kyushu University Press and S. Karger AG; 2000:67–77.Google Scholar
  16. 16.
    Sohn TA, Yeo CJ, Cameron JL, et al.: Intraductal papillary mucinous neoplasms of the pancreas: an increasingly recognized clinicopathologic entity. Ann Surg 2001, 234:313–321. The authors show that the distinct clinical features, the presumably long in situ or noninvasive phase, and the good long-term survival of patients with IPMTs offer a unique opportunity for early diagnosis, curative resection, and further studies of the molecular genetics and natural history of these unusual neoplasms.PubMedCrossRefGoogle Scholar
  17. 17.
    Falconi M, Salvia R, Bassi C, et al.: Clinicopathological features and treatment of intraductal papillary mucinous tumours of the pancreas. Br J Surg 2001, 88:376–381. Based on a study of 51 patients, resection is the treatment of choice for IPMTs. Management of the resection margin is crucial in avoiding tumor recurrence.PubMedCrossRefGoogle Scholar
  18. 18.
    Siech M, Tripp K, Schmidt-Rohlfing B, et al.: Intraductal papillary mucinous tumor of the pancreas. Am J Surg 1999, 177:117–120.PubMedCrossRefGoogle Scholar
  19. 19.
    Navarro F, Michel J, Bauret P, et al.: Management of intraductal papillary mucinous tumours of the pancreas. Eur J Surg 1999, 165:43–48.PubMedGoogle Scholar
  20. 20.
    Yamaguchi K, Yokohata K, Noshiro H, et al.: Mucinous cystic neoplasm of the pancreas or intraductal papillary-mucinous tumour of the pancreas. Eur J Surg 2000, 166:141–148.PubMedCrossRefGoogle Scholar
  21. 21.
    Izuishi K, Nakagohri T, Konishi M, et al.: Spatial assessment by magnetic resonance cholangiopancreatography for preoperative imaging in partial pancreatic head resection. Am J Surg 2001, 182:188–191.PubMedCrossRefGoogle Scholar
  22. 22.
    Wakabayashi T, Kawaura Y, Morimoto H, et al.: Clinical management of intraductal papillary mucinous tumors of the pancreas based on imaging findings. Pancreas 2001, 22:370–377.PubMedCrossRefGoogle Scholar
  23. 23.
    Kubo H, Chijiiwa Y, Akahoshi K, et al.: Intraductal papillarymucinous tumors of the pancreas: differential diagnosis between benign and malignant tumors by endoscopic ultrasonography. Am J Gastroenterol 2001, 96:1429–1434. The authors investigate signs of malignancy in duct size and branch duct tumors via EUS. They suggest that EUS is a useful modality to differentiate between benign and malignant IPMTs.PubMedCrossRefGoogle Scholar
  24. 24.
    Hara T, Yamaguchi T, Ishihara T, et al.: Diagnosis and patient management of intraductal papillary-mucinous tumor of the pancreas by using peroral pancreatoscopy and intraductal ultrasonography. Gastroenterology 2002, 122:34–43.PubMedCrossRefGoogle Scholar
  25. 25.
    Wilentz RE, Iacobuzio-Donahue CA, Argani P, et al.: Loss of expression of Dpc4 in pancreatic intraepithelial neoplasia: evidence that DPC4 inactivation occurs late in neoplastic progression. Cancer Res 2000, 60:2002–2006.PubMedGoogle Scholar
  26. 26.
    Adsay NV, Pierson C, Sarkar F, et al.: Colloid (mucinous noncystic) carcinoma of the pancreas. Am J Surg Pathol 2001, 25:26–42.PubMedCrossRefGoogle Scholar
  27. 27.
    Sugiyama M, Atomi Y: Intraductal papillary mucinous tumors of the pancreas: imaging studies and treatment strategies. Ann Surg 1998, 228:685–691.PubMedCrossRefGoogle Scholar
  28. 28.
    Z’graggen K, Rivera JA, Compton CC, et al.: Prevalence of activating K-ras mutations in the evolutionary stages of neoplasia in intraductal papillary mucinous tumors of the pancreas. Ann Surg 1997, 226:491–498. This study was the first to use IPMTs and their evolutionary stages of neoplasia for the analysis of genetic mutations. It demonstrates that IPMT is a model for pancreatic carcinogenesis.PubMedCrossRefGoogle Scholar
  29. 29.
    Kawahira H, Kobayashi S, Kaneko K, et al.: p53 protein expression in intraductal papillary mucinous tumors (IPMT) of the pancreas as an indicator of tumor malignancy. Hepatogastroenterology 2000, 47:973–977.PubMedGoogle Scholar
  30. 30.
    Kaino M, Kondoh S, Okita S, et al.: Detection of K-ras and p53 gene mutations in pancreatic juice for the diagnosis of intraductal papillary mucinous tumors. Pancreas 1999, 18:294–299.PubMedCrossRefGoogle Scholar
  31. 31.
    Iacobuzio-Donahue CA, Klimstra DS, et al.: Dpc-4 protein is expressed in virtually all human intraductal papillary mucinous neoplasms of the pancreas: comparison with conventional ductal adenocarcinomas. Am J Pathol 2000, 157:755–761. Differences in DPC4 expression between IPMTs and ductal adenocarcinomas are shown in this paper, suggesting a fundamental genetic difference in tumorigenesis that may relate to the significantly better clinical outcomes observed for IPMTs.PubMedGoogle Scholar
  32. 32.
    Whang EE, Danial T, Dunn JC, et al.: The spectrum of mucinproducing adenocarcinoma of the pancreas. Pancreas 2000, 21:147–151.PubMedCrossRefGoogle Scholar
  33. 33.
    Adsay NV, Longnecker DS, Klimstra DS: Pancreatic tumors with cystic dilatation of the ducts: intraductal papillary mucinous neoplasms and intraductal oncocytic papillary neoplasms. Semin Diagn Pathol 2000, 17:16–30.PubMedGoogle Scholar
  34. 34.
    Yonezawa S, Horinouchi M, Osako M, et al.: Gene expression of gastric type mucin (MUC5AC) in pancreatic tumors: its relationship with the biological behavior of the tumor. Pathol Int 1999, 49:45–54.PubMedCrossRefGoogle Scholar
  35. 35.
    Nakamura A, Horinouchi M, Goto M, et al.: New classification of pancreatic intraductal papillary-mucinous tumour by mucin expression: its relationship with potential for malignancy. J Pathol 2002, 197:201–210.PubMedCrossRefGoogle Scholar
  36. 36.
    Sho M, Nakajima Y, Kanehiro H, et al.: Pattern of recurrence after resection for intraductal papillary mucinous tumors of the pancreas. World J Surg 1998, 22:874–878.PubMedCrossRefGoogle Scholar
  37. 37.
    Cuillerier E, Cellier C, Palazzo L, et al.: Outcome after surgical resection of intraductal papillary and mucinous tumors of the pancreas. Am J Gastroenterol 2000, 95:441–445.PubMedCrossRefGoogle Scholar
  38. 38.
    Inoue H, Tsuchida A, Kawasaki Y, et al.: Preoperative diagnosis of intraductal papillary-mucinous tumors of the pancreas with attention to telomerase activity. Cancer 2001, 91:35–41.PubMedCrossRefGoogle Scholar
  39. 39.
    Büchler P, Conejo-Garcia JR, Lehmann G, et al.: Real-time quantitative PCR of telomerase in RNA is useful for the differentiation of benign and malignant pancreatic disorders. Pancreas 2001, 22:331–340.PubMedCrossRefGoogle Scholar
  40. 40.
    Pedrazzoli S, Beger HG, Obertop H, et al.: A surgical and pathological based classification of resective treatment of pancreatic cancer: summary of an international workshop on surgical procedures in pancreatic cancer. Dig Surg 1999, 16:337–345.PubMedCrossRefGoogle Scholar
  41. 41.
    Wurzer H, Brandstatter G, Worm HC, Wolf G: Intraductal papillary-mucinous tumors of the pancreas: presentation of eight cases. Wien Klin Wochenschr 2001, 113:880–884.PubMedGoogle Scholar
  42. 42.
    Inagaki M, Maguchi M, Kino S, et al.: Mucin-producing tumors of the pancreas: clinicopathological features, surgical treatment, and outcome. J Hepatobiliary Pancreat Surg 1999, 6:281–285.PubMedCrossRefGoogle Scholar
  43. 43.
    Seiler CA, Wagner M, Sadowski C, Kulli C, Buchler MW: Randomized prospective trial of pylorus-preserving vs. classic duodenopancreatectomy (Whipple procedure): initial clinical results. J Gastrointest Surg 2000, 4:443–452.PubMedCrossRefGoogle Scholar
  44. 44.
    Buchler MW, Friess H, Wagner M, et al.: Pancreatic fistula after pancreatic head resection. Br J Surg 2000, 87:883–889.PubMedCrossRefGoogle Scholar
  45. 45.
    Fernandez-del Castillo C: Surgical treatment of intraductal papillary mucinous neoplasms of the pancreas: the conservative approach. J Gastrointest Surg 2002, 6:660–661.PubMedCrossRefGoogle Scholar
  46. 46.
    Sarr MG, Kendrick ML, Nagorney DM, et al.: Cystic neoplasms of the pancreas: benign to malignant epithelial neoplasms. Surg Clin North Am 2001, 81:497–509.PubMedCrossRefGoogle Scholar
  47. 47.
    Sugiura H, Kondo S, Islam HK, et al.: Clinicopathologic features and outcomes of intraductal papillary-mucinous tumors of the pancreas. Hepatogastroenterology 2002, 49:263–267.PubMedGoogle Scholar
  48. 48.
    Kanazumi N, Nakao A, Kaneko T, et al.: Surgical treatment of intraductal papillary-mucinous tumors of the pancreas. Hepatogastroenterology 2001, 48:967–971.PubMedGoogle Scholar
  49. 49.
    Terris B, Ponsot P, Paye F, et al.: Intraductal papillary mucinous tumors of the pancreas confined to secondary ducts show less aggressive pathologic features as compared with those involving the main pancreatic duct. Am J Surg Pathol 2000, 24:1372–1377.PubMedCrossRefGoogle Scholar
  50. 50.
    Nakagohri T, Kenmochi T, Kainuma O, Tokoro Y, Asano T: Intraductal papillary mucinous tumors of the pancreas. Am J Surg 1999, 178:344–347.PubMedCrossRefGoogle Scholar
  51. 51.
    Chari ST, Yadav D, Smyrk TC, et al.: Study of recurrence after surgical resection of intraductal papillary mucinous neoplasm of the pancreas. Gastroenterology 2002, 123:1500–1507. This paper shows that invasive IPMTs recur frequently even after a complete "curative" resection, and that they portend poor survival. In contrast, noninvasive intraductal papillary mucinous neoplasms recur infrequently after resection, and survival is excellent regardless of the degree of epithelial dysplasia in the tumor.PubMedCrossRefGoogle Scholar
  52. 52.
    Sarr MG, Carpenter HA, Prabhakar LP, et al.: Clinical and pathologic correlation of 84 mucinous cystic neoplasms of the pancreas: can one reliably differentiate benign from malignant (or premalignant) neoplasms? Ann Surg 2000, 231:205–212.PubMedCrossRefGoogle Scholar
  53. 53.
    Nakagohri T, Asano T, Kenmochi T, et al. T: Long-term surgical outcome of noninvasive and minimally invasive intraductal papillary mucinous adenocarcinoma of the pancreas. World J Surg 2002, 26:1166–1169.PubMedCrossRefGoogle Scholar
  54. 54.
    Maire F, Hammel P, Terris B, et al.: Prognosis of malignant intraductal papillary mucinous tumours of the pancreas after surgical resection: comparison with pancreatic ductal adenocarcinoma. Gut 2002, 51:717–722.PubMedCrossRefGoogle Scholar
  55. 55.
    Loftus EV Jr, Olivares-Pakzad BA, Batts KP, et al.: Intraductal papillary-mucinous tumors of the pancreas: clinicopathologic features, outcome, and nomenclature. Members of the Pancreas Clinic and Pancreatic Surgeons of Mayo Clinic. Gastroenterology 1996,110:1909–1918.CrossRefGoogle Scholar

Copyright information

© Current Science Inc. 2003

Authors and Affiliations

  • Friedrich H. Schmitz-Winnenthal
  • Kaspar Z’graggen
  • Christine Volk
  • Bruno M. Schmied
  • Markus W. Büchler
    • 1
  1. 1.Department of General SurgeryRuprecht-Karls-University HeidelbergHeidelbergGermany

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