Skip to main content

Intraduktale Pankreasneoplasien

Zystisch und häufig

Intraductal neoplasms of the pancreas: cystic and common


Der wichtigste Vertreter der insgesamt seltenen intraduktalen Tumoren des Pankreas ist die intraduktale papillär-muzinöse Neoplasie (IPMN). Dieser Tumor, der erst in den letzten Jahren im Detail beschrieben wurde, ist 1996 in die WHO-Klassifikation aufgenommen worden. Die IPMN zeigen für lange Zeit ein intraduktales Wachstum, bevor sie in etwa 50% der Fälle invasiv werden. Damit grenzen sie sich prognostisch deutlich vom duktalen Adenokarzinom ab. Gegenwärtig sind die IPMN die häufigsten zystischen Neoplasien, die reseziert werden. Neue Untersuchungen zeigen, dass die IPMN aufgrund ihres Muzin-Musters in verschiedene Typen mit wahrscheinlich unterschiedlicher Biologie unterteilt werden müssen.


The most important of the generally rare intraductal tumors of the pancreas is the intraductal papillary-mucinous neoplasm (IPMN). In recent years this tumor type has been described in detail, and it was included in the WHO classification of 1996. IPMNs typically grow for a long time within the ducts, before approximately 50% of them eventually become invasive. Because of this feature the IPMNs are clearly distinct from ductal adenocarcinomas. Currently, IPMNs have advanced to the most commonly resected cystic tumor of the pancreas. Recent studies have shown that IPMNs may be distinguished into different types showing a different biology according to their mucin pattern.

This is a preview of subscription content, access via your institution.

Abb. 1
Abb. 2
Abb. 3


  1. 1.

    Abraham SC, Lee JH, Boitnott JK et al. (2002) Microsatellite instability in intraductal papillary neoplasms of the biliary tract. Mod Pathol 15:1309–1317

    Article  PubMed  Google Scholar 

  2. 2.

    Adsay NV, Adair CF, Heffess CS, Klimstra DS (1996) Intraductal oncocytic papillary neoplasms of the pancreas. Am J Surg Pathol 20:980–994

    Article  CAS  PubMed  Google Scholar 

  3. 3.

    Adsay NV, Conlon KC, Zee SY et al. (2002) Intraductal papillary-mucinous neoplasms of the pancreas. An analysis of in situ and invasive carcinomas in 28 patients. Cancer 94:62–77

    Article  PubMed  Google Scholar 

  4. 4.

    Adsay NV, Hasteh F, Cheng JD et al. (2002) Lymphoepithelial cysts of the pancreas: a report of 12 cases and a review of the literature. Mod Pathol 15:492–501

    Article  PubMed  Google Scholar 

  5. 5.

    Adsay NV, Merati K, Basturk O et al. (2004) Pathologically and biologically distinct types of epithelium in intraductal papillary mucinous neoplasms. Delineation of an „intestinal“ pathway of carcinogenesis in the pancreas. Am J Surg Pathol 28:839–848

    PubMed  Google Scholar 

  6. 6.

    Albores-Saavedra J, Sheahan K, O’Riain C, Shukla D (2004) Intraductal tubular adenoma, pyloric type, of the pancreas. Additional observations on a new type of pancreatic neoplasm. Am J Surg Pathol 28:233–238

    PubMed  Google Scholar 

  7. 7.

    Azar C, Van de Stadt J, Rickaert F et al. (1996) Intraductal papillary mucinous tumours of the pancreas. Clinical and therapeutic issues in 32 patients. Gut 39:457–464

    CAS  PubMed  Google Scholar 

  8. 8.

    Biankin AV, Biankin SA, Kench JG et al. (2002) Aberrant p16(INK4A) and DPC4/Smad4 expression in intraductal papillary mucinous tumours of the pancreas is associated with invasive ductal adenocarcinoma. Gut 50:861–868

    Article  CAS  PubMed  Google Scholar 

  9. 9.

    Chari ST, Yadav D, Smyrk TC et al. (2002) Study of recurrence after surgical resection of intraductal papillary mucinous neoplasm of the pancreas. Gastroenterology 123:1500–1507

    PubMed  Google Scholar 

  10. 10.

    Chen TC, Nakanuma Y, Zen Y et al. (2001) Intraductal papillary neoplasia of the liver associated with hepatolithiasis. Hepatology 34:651–658

    Article  CAS  PubMed  Google Scholar 

  11. 11.

    Esposito I, Bauer A, Hoheisel JD et al. (2004) Microcystic tubulopapillary carcinoma of the pancreas: a new tumor entity? Virchows Arch 444:447–453

    Article  PubMed  Google Scholar 

  12. 12.

    Fabre A, Sauvanet A, Fléjou JF et al. (2001) Intraductal acinar cell carcinoma of the pancreas. Virchows Arch 438:312–315

    Article  CAS  PubMed  Google Scholar 

  13. 13.

    Fujii H, Inagaki M, Kasai S et al. (1997) Genetic progression and heterogeneity in intraductal papillary-mucinous neoplasms of the pancreas. Am J Pathol 151:1447–1454

    CAS  PubMed  Google Scholar 

  14. 14.

    Iacobuzio-Donahue CA, Wilentz RE, Argani P et al. (2000) Dpc4 protein in mucinous cystic neoplasms of the pancreas. Frequent loss of expression in invasive carcinomas suggests a role in genetic progression. Am J Surg Pathol 24:1544–1548

    CAS  PubMed  Google Scholar 

  15. 15.

    Inoue H, Tsuchida A, Kawasaki Y et al. (2001) Preoperative diagnosis of intraductal papillary-mucinous tumors of the pancreas with attention to telomerase activity. Cancer 91:35–41

    Article  CAS  PubMed  Google Scholar 

  16. 16.

    Kato N, Akiyama S, Motoyama T (2002) Pyloric gland-type tubular adenoma superimposed on intraductal papillary mucinous tumor of the pancreas. Pyloric gland adenoma of the pancreas. Virchows Arch 440:205–208

    Article  PubMed  Google Scholar 

  17. 17.

    Klöppel G, Solcia E, Longnecker DS et al. (1996) Histological typing of tumours of the exocrine pancreas, 2nd edn. WHO International histological classification of tumours. Springer, Berlin Heidelberg New York Tokyo

  18. 18.

    Kosmahl M, Pauser U, Peters K et al. (2004) Cystic neoplasms of the pancreas and tumor-like lesions with cystic features: a review of 418 cases and a classification proposal. Virchows Arch 445:168–178

    Article  CAS  PubMed  Google Scholar 

  19. 19.

    Loftus EV Jr, Olivares-Pakzad BA, Batts KP et al., Members of the Pancreas Clinic, Pancreatic Surgeons of Mayo Clinic (1996) Intraductal papillary-mucinous tumors of the pancreas: clinicopathologic features, outcome, and nomenclature. Gastroenterology 110:1909–1918

    PubMed  Google Scholar 

  20. 20.

    Lüttges J, Beyser K, Pust S et al. (2003) Pancreatic mucinous noncystic (colloid) carcinomas and intraductal papillary mucinous carcinomas are usually microsatellite stable. Mod Pathol 16:537–542

    Article  PubMed  Google Scholar 

  21. 21.

    Lüttges J, Feyerabend B, Buchelt T et al. (2002) The mucin profile of noninvasive and invasive mucinous cystic neoplasms of the pancreas. Am J Surg Pathol 26:466–471

    Article  PubMed  Google Scholar 

  22. 22.

    Lüttges J, Zamboni G, Longnecker D, Klöppel G (2001) The immunohistochemical mucin expression pattern distinguishes different types of intraductal papillary mucinous neoplasms of the pancreas and determines their relationship to mucinous noncystic carcinoma and ductal adenocarcinoma. Am J Surg Pathol 25:942–948

    CAS  PubMed  Google Scholar 

  23. 23.

    Maire F, Hammel P, Terris B et al. (2002) Prognosis of malignant intraductal papillary mucinous tumours of the pancreas after surgical resection. Comparison with pancreatic ductal adenocarcinoma. Gut 51:717–722

    Article  CAS  PubMed  Google Scholar 

  24. 24.

    Mills SE, Carter D, Greenson JK et al. (2004) Sternberg’s diagnostic surgical pathology. Lippincott Williams & Wilkins, Philadelphia

  25. 25.

    Nakagohri T, Asano T, Kenmochi T et al. (2002) Long-term surgical outcome of noninvasive and minimally invasive intraductal papillary mucinous adenocarcinoma of the pancreas. World J Surg 26:1166–1169

    Article  PubMed  Google Scholar 

  26. 26.

    Nakamura A, Horinouchi M, Goto M et al. (2002) New classification of pancreatic intraductal papillary-mucinous tumour by mucin expression: its relationship with potential for malignancy. J Pathol 197:201–210

    Article  PubMed  Google Scholar 

  27. 27.

    Paye F, Sauvanet A, Terris B et al. (2000) Intraductal papillary mucinous tumors of the pancreas: pancreatic resections guided by preoperative morphological assessment and intraoperative frozen section examination. Surgery 127:536–544

    Article  CAS  PubMed  Google Scholar 

  28. 28.

    Remmele W (1997) Pathologie, Bd 3.2., neubearb. Aufl. Springer, Berlin Heidelberg New York Tokyo

  29. 29.

    Rosai J (2004) Rosai and Ackerman’s surgical pathology, vol 1.2., 9th edn. Mosby, Edinburgh

  30. 30.

    Salvia R, Fernandez-del Castillo C, Bassi C et al. (2004) Main-duct intraductal papillary mucinous neoplasms of the pancreas: clinical predictors of malignancy and long-term survival following resection. Ann Surg 239:678–685

    Article  PubMed  Google Scholar 

  31. 31.

    Sato N, Rosty C, Jansen M et al. (2001) STK11/LKB1 Peutz-Jeghers gene inactivation in intraductal papillary-mucinous neoplasms of the pancreas. Am J Pathol 159:2017–2022

    CAS  PubMed  Google Scholar 

  32. 32.

    Sessa F, Solcia E, Capella C et al. (1994) Intraductal papillary-mucinous tumours represent a distinct group of pancreatic neoplasms: an investigation of tumour cell differentiation and K-ras, p53, and c-erbB-2 abnormalities in 26 patients. Virchows Arch 425:357–367

    Article  CAS  PubMed  Google Scholar 

  33. 33.

    Shibahara H, Tamada S, Goto M et al. (2004) Pathologic features of mucin-producing bile duct tumors: two histopathologic categories as counterparts of pancreatic intraductal papillary-mucinous neoplasms. Am J Surg Pathol 28:327–338

    PubMed  Google Scholar 

  34. 34.

    Sohn TA, Yeo CJ, Cameron JL et al. (2004) Intraductal papillary mucinous neoplasms of the pancreas: an updated experience. Ann Surg 239:788–797

    Article  PubMed  Google Scholar 

  35. 35.

    Soldini D, Gugger M, Burckhardt E et al. (2003) Progressive genomic alterations in intraductal papillary mucinous tumours of the pancreas and morphologically similar lesions of the pancreatic ducts. J Pathol 199:453–461

    Article  CAS  PubMed  Google Scholar 

  36. 36.

    Sugiyama M, Izumisato Y, Abe N et al. (2003) Predictive factors for malignancy in intraductal papillary-mucinous tumours of the pancreas. Br J Surg 90:1244–1249

    Article  CAS  PubMed  Google Scholar 

  37. 37.

    Terris B, Ponsot T, Paye F et al. (2000) 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 24:1372–1377

    CAS  PubMed  Google Scholar 

  38. 38.

    Wada K (2002) p16 and p53 gene alterations and accumulations in the malignant evolution of intraductal papillary-mucinous tumors of the pancreas. J Hepatobiliary Pancreat Surg 9:76–85

    Article  PubMed  Google Scholar 

  39. 39.

    Z’Graggen K, Rivera JA, Compton CC et al. (1997) Prevalence of activating K-ras mutations in the evolutionary stages of neoplasia in intraductal papillary mucinous tumors of the pancreas. Ann Surg 226:491–498

    Article  PubMed  Google Scholar 

  40. 40.

    Zamboni G, Scarpa A, Bogina G et al. (1999) Mucinous cystic tumors of the pancreas. Clinicopathological features, prognosis and relationship to other mucinous cystic tumors. Am J Surg Pathol 23:410–422

    Article  CAS  PubMed  Google Scholar 

Download references


Keine Angaben

Author information



Corresponding author

Correspondence to G. Klöppel.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Klöppel, G., Kosmahl, M. & Lüttges, J. Intraduktale Pankreasneoplasien. Pathologe 26, 31–36 (2005).

Download citation


  • Intraduktale papillär-muzinöse Neoplasie (IPMN)
  • Intestinaler Typ
  • Pankreatobiliärer Typ
  • Pathologie
  • Immunhistochemie


  • Intraductal papillary-mucinous neoplasm
  • Intestinal type
  • Pancreatobiliary type
  • Pathology
  • Immunohistochemistry