Skip to main content
SpringerLink
Log in

Intraductal Papillary Mucinous Neoplasms

  • Chapter
  • First Online:
Pancreas and Biliary Tract Cytohistology

Part of the book series: Essentials in Cytopathology ((EICP,volume 28))

  • 698 Accesses

Abstract

With ever-increasing use of cross-sectional abdominal imaging, there has been a rise in the number of asymptomatic pancreatic cysts that are detected incidentally, the management of which poses a major issue. A large proportion of these asymptomatic pancreatic cysts are believed to comprise of branch duct intraductal papillary mucinous neoplasms (IPMNs). Depending upon the involvement of the branch ducts and/or main pancreatic duct, IPMNs can be subdivided into branch duct IPMN, main duct IPMN, and mixed- or combined-type IPMN. Main duct IPMNs are associated with the highest rate of malignancy (mean frequency, 61.6%). Four distinct histologic subtypes of IPMNs are recognized based on the type of epithelial lining: gastric, intestinal, pancreatobiliary, and oncocytic. Based on the highest degree of architectural and cytologic atypia, the epithelial dysplasia is graded as low, intermediate, and high. The preoperative diagnosis of IPMNs is based on a multimodal approach comprising of clinical and imaging findings and cyst fluid analysis with cytologic examination. This chapter will discuss various aspects of IPMNs including the clinical features, imaging findings, pathologic features, molecular genetics, and management recommendations.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 99.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Laffan TA, Horton KM, Klein AP, Berlanstein B, Siegelman SS, Kawamoto S, et al. Prevalence of unsuspected pancreatic cysts on MDCT. AJR Am J Roentgenol. 2008;191:802–7. https://doi.org/10.2214/AJR.07.3340.

    Article  PubMed  PubMed Central  Google Scholar 

  2. de Jong K, Nio CY, Hermans JJ, Dijkgraaf MG, Gouma DJ, van Eijck CH. High prevalence of pancreatic cysts detected by screening magnetic resonance imaging examinations. Clin Gastroenterol Hepatol. 2010;8:806–11. https://doi.org/10.1016/j.cgh.2010.05.017.

    Article  PubMed  Google Scholar 

  3. Lee KS, Sekhar A, Rofsky NM, Pedrosa I. Prevalence of incidental pancreatic cysts in the adult population on MR imaging. Am J Gastroenterol. 2010;105:2079–84. https://doi.org/10.1038/ajg.2010.122.

    Article  PubMed  Google Scholar 

  4. Morales-Oyarvide V, Fong ZV, Fernández-Del Castillo C, Warshaw AL. Intraductal papillary mucinous neoplasms of the pancreas: strategic considerations. Visc Med. 2017;33:466–76. https://doi.org/10.1159/000485014.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Pergolini I, Sahora K, Ferrone CR, Morales-Oyarvide V, Wolpin BM, Mucci LA, et al. Long-term risk of pancreatic malignancy in patients with branch duct intraductal papillary mucinous neoplasm in a Referral Center. Gastroenterology. 2017;153:1284–1294.e1. https://doi.org/10.1053/j.gastro.2017.07.019.

    Article  PubMed  Google Scholar 

  6. Morales-Oyarvide V, Mino-Kenudson M, Ferrone CR, Sahani DV, Pergolini I, Negreros-Osuna AA, et al. Diabetes mellitus in intraductal papillary mucinous neoplasm of the pancreas is associated with high-grade dysplasia and invasive carcinoma. Pancreatology. 2017;17:920–6. https://doi.org/10.1016/j.pan.2017.08.073.

    Article  PubMed  Google Scholar 

  7. Tanaka M, Fernández-Del Castillo C, Kamisawa T, Jang JY, Levy P, Ohtsuka T, et al. Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas. Pancreatology. 2017;17:738–53. https://doi.org/10.1016/j.pan.2017.07.007.

    Article  PubMed  Google Scholar 

  8. Berland LL, Silverman SG, Gore RM, Mayo-Smith WW, Megibow AJ, Yee J, et al. Managing incidental findings on abdominal CT: white paper of the ACR incidental findings committee. J Am Coll Radiol. 2010;7:754–73. https://doi.org/10.1016/j.jacr.2010.06.013.

    Article  PubMed  Google Scholar 

  9. Efthymiou A, Podas T, Zacharakis E. Endoscopic ultrasound in the diagnosis of pancreatic intraductal papillary mucinous neoplasms. World J Gastroenterol. 2014;20:7785–93. https://doi.org/10.3748/wjg.v20.i24.7785.

    Article  PubMed  PubMed Central  Google Scholar 

  10. Kitano M, Sakamoto H, Komaki T, Kudo M. New techniques and future perspective of EUS for the differential diagnosis of pancreatic malignancies: contrast harmonic imaging. Dig Endosc. 2011;23(Suppl 1):46–50. https://doi.org/10.1111/j.1443–1661.2011.01146.x.

    Article  PubMed  Google Scholar 

  11. Tanaka M, Fernández-del Castillo C, Adsay V, Chari S, Falconi M, Jang JY, et al. International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology. 2012;12:183–97. https://doi.org/10.1016/j.pan.2012.04.004.

    Article  PubMed  Google Scholar 

  12. Adsay NV, Fukushima N, Furukawa T, Hruban RH, Klimstra DS, Klöppel G, et al. Intraductal neoplasms of the pancreas. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. WHO classification of tumors of the digestive system. 4th ed. Lyon: IARC; 2010. p. 304–13.

    Google Scholar 

  13. Pitman MB, Lewandrowski K, Shen J, Sahani D, Brugge W, Fernandez-del Castillo C. Pancreatic cysts: preoperative diagnosis and clinical management. Cancer Cytopathol. 2010;118:1–13. https://doi.org/10.1002/cncy.20059.

    Article  PubMed  Google Scholar 

  14. Smith AL, Abdul-Karim FW, Goyal A. Cytologic categorization of pancreatic neoplastic mucinous cysts with an assessment of the risk of malignancy: a retrospective study based on the Papanicolaou Society of Cytopathology guidelines. Cancer Cytopathol. 2016;124:285–93. https://doi.org/10.1002/cncy.21657.

    Article  PubMed  Google Scholar 

  15. Layfield LJ, Cramer H. Fine-needle aspiration cytology of intraductal papillary-mucinous tumors: a retrospective analysis. Diagn Cytopathol. 2005;32:16–20.

    Article  Google Scholar 

  16. Stelow EB, Stanley MW, Bardales RH, Mallery S, Lai R, Linzie BM, et al. Intraductal papillary-mucinous neoplasm of the pancreas. The findings and limitations of cytologic samples obtained by endoscopic ultrasound-guided fine-needle aspiration. Am J Clin Pathol. 2003;120:398–404.

    Article  Google Scholar 

  17. Michaels PJ, Brachtel EF, Bounds BC, Brugge WR, Pitman MB. Intraductal papillary mucinous neoplasm of the pancreas: cytologic features predict histologic grade. Cancer. 2006;108:163–73.

    Article  Google Scholar 

  18. Pitman MB, Genevay M, Yaeger K, Chebib I, Turner BG, Mino-Kenudson M, et al. High-grade atypical epithelial cells in pancreatic mucinous cysts are a more accurate predictor of malignancy than “positive” cytology. Cancer Cytopathol. 2010;118:434–40. https://doi.org/10.1002/cncy.20118.

    Article  PubMed  Google Scholar 

  19. Pitman MB, Layfield LJ. Guidelines for pancreaticobiliary cytology from the Papanicolaou Society of Cytopathology: a review. Cancer Cytopathol. 2014;122:399–411. https://doi.org/10.1002/cncy.21427.

    Article  PubMed  Google Scholar 

  20. Pitman MB, Centeno BA, Genevay M, Fonseca R, Mino-Kenudson M. Grading epithelial atypia in endoscopic ultrasound-guided fine-needle aspiration of intraductal papillary mucinous neoplasms: an international interobserver concordance study. Cancer Cytopathol. 2013;121:729–36. https://doi.org/10.1002/cncy.21334.

    Article  PubMed  Google Scholar 

  21. Lee PJ, Fischer AH, Owens CL, Hutchinson L. Intranuclear cytoplasmic inclusions are a specific diagnostic feature distinguishing low-grade pancreatic intraductal papillary mucinous neoplasms from contaminating gastric epithelium. J Am Soc Cytopathol. 2013;2:S66–7. https://doi.org/10.1016/j.jasc.2013.08.182.

    Article  Google Scholar 

  22. Pitman MB, Centeno BA, Daglilar ES, Brugge WR, Mino-Kenudson M. Cytological criteria of high-grade epithelial atypia in the cyst fluid of pancreatic intraductal papillary mucinous neoplasms. Cancer Cytopathol. 2014;122:40–7. https://doi.org/10.1002/cncy.21344.

    Article  PubMed  Google Scholar 

  23. Sigel CS, Edelweiss M, Tong LC, Magda J, Oen H, Sigel KM, et al. Low interobserver agreement in cytology grading of mucinous pancreatic neoplasms. Cancer Cytopathol. 2015;123:40–50. https://doi.org/10.1002/cncy.21492.

    Article  CAS  PubMed  Google Scholar 

  24. Goyal A, Abdul-Karim FW, Yang B, Patel JB, Brainard JA. Interobserver agreement in the cytologic grading of atypia in neoplastic pancreatic mucinous cysts with the 2-tiered approach. Cancer Cytopathol. 2016;124:909–16. https://doi.org/10.1002/cncy.21767.

    Article  CAS  PubMed  Google Scholar 

  25. Zhang ML, Arpin RN, Brugge WR, Forcione DG, Basar O, Pitman MB. Moray micro forceps biopsy improves the diagnosis of specific pancreatic cysts. Cancer Cytopathol. 2018;126:414–20. https://doi.org/10.1002/cncy.21988.

    Article  CAS  PubMed  Google Scholar 

  26. Brugge WR, Lewandrowski K, Lee-Lewandrowski E, Centeno BA, Szydlo T, Regan S, et al. Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study. Gastroenterology. 2004;126:1330–6.

    Article  Google Scholar 

  27. Cizginer S, Turner BG, Bilge AR, Karaca C, Pitman MB, Brugge WR. Cyst fluid carcinoembryonic antigen is an accurate diagnostic marker of pancreatic mucinous cysts. Pancreas. 2011;40:1024–8. https://doi.org/10.1097/MPA.0b013e31821bd62f.

    Article  CAS  PubMed  Google Scholar 

  28. Rockacy M, Khalid A. Update on pancreatic cyst fluid analysis. Ann Gastroenterol. 2013;26:122–7.

    PubMed  PubMed Central  Google Scholar 

  29. Oh HC, Kang H, Brugge WR. Cyst fluid amylase and CEA levels in the differential diagnosis of pancreatic cysts: a single-center experience with histologically proven cysts. Dig Dis Sci. 2014;59:3111–6. https://doi.org/10.1007/s10620–014–3254–8.

    Article  PubMed  Google Scholar 

  30. Reid MD, Saka B, Balci S, Goldblum AS, Adsay NV. Molecular genetics of pancreatic neoplasms and their morphologic correlates: an update on recent advances and potential diagnostic applications. Am J Clin Pathol. 2014;141:168–80. https://doi.org/10.1309/AJCP0FKDP7ENVKEV.

    Article  CAS  PubMed  Google Scholar 

  31. Singhi AD, Nikiforova MN, Fasanella KE, McGrath KM, Pai RK, Ohori NP, et al. Preoperative GNAS and KRAS testing in the diagnosis of pancreatic mucinous cysts. Clin Cancer Res. 2014;20:4381–9. https://doi.org/10.1158/1078–0432.CCR-14–0513.

    Article  CAS  PubMed  Google Scholar 

  32. Wu J, Matthaei H, Maitra A, Dal Molin M, Wood LD, Eshleman JR, et al. Recurrent GNAS mutations define an unexpected pathway for pancreatic cyst development. Sci Transl Med. 2011;3:92ra66. https://doi.org/10.1126/scitranslmed.3002543.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Kadayifci A, Atar M, Wang JL, Forcione DG, Casey BW, Pitman MB, et al. Value of adding GNAS testing to pancreatic cyst fluid KRAS and carcinoembryonic antigen analysis for the diagnosis of intraductal papillary mucinous neoplasms. Dig Endosc. 2017;29:111–7. https://doi.org/10.1111/den.12710.

    Article  PubMed  Google Scholar 

  34. Lee LS, Doyle LA, Houghton J, Sah S, Bellizzi AM, Szafranska-Schwarzbach AE, et al. Differential expression of GNAS and KRAS mutations in pancreatic cysts. JOP. 2014;15:581–6. https://doi.org/10.6092/1590–8577/2432.

    Article  PubMed  Google Scholar 

  35. Springer S, Wang Y, Dal Molin M, Masica DL, Jiao Y, Kinde I, et al. A combination of molecular markers and clinical features improve the classification of pancreatic cysts. Gastroenterology. 2015;149:1501–10. https://doi.org/10.1053/j.gastro.2015.07.041.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Jones M, Zheng Z, Wang J, Dudley J, Albanese E, Kadayifci A, et al. Impact of next-generation sequencing on the clinical diagnosis of pancreatic cysts. Gastrointest Endosc. 2016;83:140–8. https://doi.org/10.1016/j.gie.2015.06.047.

    Article  PubMed  Google Scholar 

  37. Matthaei H, Wylie D, Lloyd MB, Dal Molin M, Kemppainen J, Mayo SC, et al. miRNA biomarkers in cyst fluid augment the diagnosis and management of pancreatic cysts. Clin Cancer Res. 2012;18:4713–24. https://doi.org/10.1158/1078–0432.CCR-12–0035.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Vege SS, Ziring B, Jain R, Moayyedi P, Clinical Guidelines Committee; American Gastroenterology Association. American gastroenterological association institute guideline on the diagnosis and management of asymptomatic neoplastic pancreatic cysts. Gastroenterology. 2015;148:819–22. ; quize12–3. https://doi.org/10.1053/j.gastro.2015.01.015.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Weill-Cornell Medicine, New York Presbyterian Hosptial, New York, NY, USA

    Lorene Yoxtheimer & Abha Goyal

Authors
  1. Lorene Yoxtheimer
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Abha Goyal
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Abha Goyal .

Editor information

Editors and Affiliations

  1. Department of Pathology and Laboratory Medicine, Weill-Cornell Medicine, NewYork Presbyterian Hospital, New York, NY, USA

    Abha Goyal

  2. Department of Pathology and Laboratory Medicine, Weill-Cornell Medicine, NewYork Presbyterian Hospital, New York, NY, USA

    Rema Rao

  3. Department of Pathology and Laboratory Medicine, Weill-Cornell Medicine, New York Presbyterian Hospital, New York, NY, USA

    Momin T. Siddiqui

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Yoxtheimer, L., Goyal, A. (2019). Intraductal Papillary Mucinous Neoplasms. In: Goyal, A., Rao, R., Siddiqui, M. (eds) Pancreas and Biliary Tract Cytohistology. Essentials in Cytopathology, vol 28. Springer, Cham. https://doi.org/10.1007/978-3-030-22433-2_7

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-22433-2_7

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-22432-5

  • Online ISBN: 978-3-030-22433-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation