Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Pancreatic cystic neoplasms: Clinical manifestations, diagnosis, and management

Asif Khalid, MD
Kevin McGrath, MD
Section Editor
John R Saltzman, MD, FACP, FACG, FASGE, AGAF
Deputy Editor
Shilpa Grover, MD, MPH, AGAF


Pancreatic cysts are diagnosed with increasing frequency because of the widespread use of cross-sectional imaging. Pancreatic cysts may be detected in over 2 percent of patients who undergo abdominal imaging with multidetector-row computed tomography or magnetic resonance imaging for unrelated reasons, and this frequency increases with age [1,2].

This topic will review issues related to the evaluation and management of pancreatic cystic neoplasms, including serous cystic tumors, mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, and solid pseudopapillary neoplasms. The classification of pancreatic cysts, the diagnosis and management of pancreatic fluid collections, and more detailed discussions of intraductal papillary mucinous neoplasms of the pancreas are discussed separately:

(See "Classification of pancreatic cysts".)

(See "Management of pancreatic pseudocysts and walled-off pancreatic necrosis".)

(See "Endoscopic management of walled-off pancreatic fluid collections: Techniques".)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Jun 16, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Laffan TA, Horton KM, Klein AP, et al. Prevalence of unsuspected pancreatic cysts on MDCT. AJR Am J Roentgenol 2008; 191:802.
  2. de Jong K, Nio CY, Hermans JJ, et al. High prevalence of pancreatic cysts detected by screening magnetic resonance imaging examinations. Clin Gastroenterol Hepatol 2010; 8:806.
  3. Spinelli KS, Fromwiller TE, Daniel RA, et al. Cystic pancreatic neoplasms: observe or operate. Ann Surg 2004; 239:651.
  4. Fernández-del Castillo C, Targarona J, Thayer SP, et al. Incidental pancreatic cysts: clinicopathologic characteristics and comparison with symptomatic patients. Arch Surg 2003; 138:427.
  5. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System, Aaltonen LA, Hamilton SR (Eds), IARC Press, Lyon, France 2000.
  6. Zamboni G, Kloeppel G, Hruban RH, et, al. Mucinous cystic neoplasms of the pancreas. In: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System, Aaltonen LA, Hamilton SR (Eds), IARC Press, Lyon, France 2000. p.234.
  7. Valsangkar NP, Morales-Oyarvide V, Thayer SP, et al. 851 resected cystic tumors of the pancreas: a 33-year experience at the Massachusetts General Hospital. Surgery 2012; 152:S4.
  8. Scheiman JM, Hwang JH, Moayyedi P. American gastroenterological association technical review on the diagnosis and management of asymptomatic neoplastic pancreatic cysts. Gastroenterology 2015; 148:824.
  9. Compagno J, Oertel JE. Microcystic adenomas of the pancreas (glycogen-rich cystadenomas): a clinicopathologic study of 34 cases. Am J Clin Pathol 1978; 69:289.
  10. Pyke CM, van Heerden JA, Colby TV, et al. The spectrum of serous cystadenoma of the pancreas. Clinical, pathologic, and surgical aspects. Ann Surg 1992; 215:132.
  11. Lundstedt C, Dawiskiba S. Serous and mucinous cystadenoma/cystadenocarcinoma of the pancreas. Abdom Imaging 2000; 25:201.
  12. Tseng JF, Warshaw AL, Sahani DV, et al. Serous cystadenoma of the pancreas: tumor growth rates and recommendations for treatment. Ann Surg 2005; 242:413.
  13. Sharma, A. Tumors of the Pancreas. In: Current Diagnosis & Treatment: Gastroenterology, Hepatology, & Endoscopy, Greenberger, NJ, Blumberg, RS, Burakoff, R (Eds), McGraw-Hill, New York 2009. p.318.
  14. Romics L Jr, Oláh A, Belágyi T, et al. Solid pseudopapillary neoplasm of the pancreas--proposed algorithms for diagnosis and surgical treatment. Langenbecks Arch Surg 2010; 395:747.
  15. Gress F, Gottlieb K, Cummings O, et al. Endoscopic ultrasound characteristics of mucinous cystic neoplasms of the pancreas. Am J Gastroenterol 2000; 95:961.
  16. 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.
  17. Zamboni G, Scarpa A, Bogina G, et al. Mucinous cystic tumors of the pancreas: clinicopathological features, prognosis, and relationship to other mucinous cystic tumors. Am J Surg Pathol 1999; 23:410.
  18. Reddy RP, Smyrk TC, Zapiach M, et al. Pancreatic mucinous cystic neoplasm defined by ovarian stroma: demographics, clinical features, and prevalence of cancer. Clin Gastroenterol Hepatol 2004; 2:1026.
  19. Machado NO, Al Qadhi H, Al Wahibi K. Intraductal Papillary Mucinous Neoplasm of Pancreas. N Am J Med Sci 2015; 7:160.
  20. Irie H, Honda H, Aibe H, et al. MR cholangiopancreatographic differentiation of benign and malignant intraductal mucin-producing tumors of the pancreas. AJR Am J Roentgenol 2000; 174:1403.
  21. Sahani DV, Kadavigere R, Blake M, et al. Intraductal papillary mucinous neoplasm of pancreas: multi-detector row CT with 2D curved reformations--correlation with MRCP. Radiology 2006; 238:560.
  22. Carbognin G, Zamboni G, Pinali L, et al. Branch duct IPMTs: value of cross-sectional imaging in the assessment of biological behavior and follow-up. Abdom Imaging 2006; 31:320.
  23. Kim JH, Eun HW, Kim KW, et al. Intraductal papillary mucinous neoplasms with associated invasive carcinoma of the pancreas: imaging findings and diagnostic performance of MDCT for prediction of prognostic factors. AJR Am J Roentgenol 2013; 201:565.
  24. Waters JA, Schmidt CM, Pinchot JW, et al. CT vs MRCP: optimal classification of IPMN type and extent. J Gastrointest Surg 2008; 12:101.
  25. Sugiyama M, Atomi Y, Kuroda A. Two types of mucin-producing cystic tumors of the pancreas: diagnosis and treatment. Surgery 1997; 122:617.
  26. Koito K, Namieno T, Ichimura T, et al. Mucin-producing pancreatic tumors: comparison of MR cholangiopancreatography with endoscopic retrograde cholangiopancreatography. Radiology 1998; 208:231.
  27. 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.
  28. Albert J, Schilling D, Breer H, et al. Mucinous cystadenomas and intraductal papillary mucinous tumors of the pancreas in magnetic resonance cholangiopancreatography. Endoscopy 2000; 32:472.
  29. Itai Y, Ohhashi K, Nagai H, et al. "Ductectatic" mucinous cystadenoma and cystadenocarcinoma of the pancreas. Radiology 1986; 161:697.
  30. Yu MH, Lee JY, Kim MA, et al. MR imaging features of small solid pseudopapillary tumors: retrospective differentiation from other small solid pancreatic tumors. AJR Am J Roentgenol 2010; 195:1324.
  31. Vege SS, Ziring B, Jain R, et al. American gastroenterological association institute guideline on the diagnosis and management of asymptomatic neoplastic pancreatic cysts. Gastroenterology 2015; 148:819.
  32. Singhi AD, Zeh HJ, Brand RE, et al. American Gastroenterological Association guidelines are inaccurate in detecting pancreatic cysts with advanced neoplasia: a clinicopathologic study of 225 patients with supporting molecular data. Gastrointest Endosc 2016; 83:1107.
  33. ASGE Standards of Practice Committee, Muthusamy VR, Chandrasekhara V, et al. The role of endoscopy in the diagnosis and treatment of cystic pancreatic neoplasms. Gastrointest Endosc 2016; 84:1.
  34. Brugge WR, Lewandrowski K, Lee-Lewandrowski E, et al. Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study. Gastroenterology 2004; 126:1330.
  35. Al-Haddad M, Gill KR, Raimondo M, et al. Safety and efficacy of cytology brushings versus standard fine-needle aspiration in evaluating cystic pancreatic lesions: a controlled study. Endoscopy 2010; 42:127.
  36. Cizginer S, Turner BG, Bilge AR, et al. Cyst fluid carcinoembryonic antigen is an accurate diagnostic marker of pancreatic mucinous cysts. Pancreas 2011; 40:1024.
  37. Gaddam S, Ge PS, Keach JW, et al. Suboptimal accuracy of carcinoembryonic antigen in differentiation of mucinous and nonmucinous pancreatic cysts: results of a large multicenter study. Gastrointest Endosc 2015; 82:1060.
  38. Khalid A, McGrath KM, Zahid M, et al. The role of pancreatic cyst fluid molecular analysis in predicting cyst pathology. Clin Gastroenterol Hepatol 2005; 3:967.
  39. Khalid A, Zahid M, Finkelstein SD, et al. Pancreatic cyst fluid DNA analysis in evaluating pancreatic cysts: a report of the PANDA study. Gastrointest Endosc 2009; 69:1095.
  40. Rockacy MJ, Zahid M, McGrath KM, et al. Association between KRAS mutation, detected in pancreatic cyst fluid, and long-term outcomes of patients. Clin Gastroenterol Hepatol 2013; 11:425.
  41. Park WG, Wu M, Bowen R, et al. Metabolomic-derived novel cyst fluid biomarkers for pancreatic cysts: glucose and kynurenine. Gastrointest Endosc 2013; 78:295.
  42. Al-Haddad M, DeWitt J, Sherman S, et al. Performance characteristics of molecular (DNA) analysis for the diagnosis of mucinous pancreatic cysts. Gastrointest Endosc 2014; 79:79.
  43. Jabbar KS, Verbeke C, Hyltander AG, et al. Proteomic mucin profiling for the identification of cystic precursors of pancreatic cancer. J Natl Cancer Inst 2014; 106:djt439.
  44. Zikos T, Pham K, Bowen R, et al. Cyst Fluid Glucose is Rapidly Feasible and Accurate in Diagnosing Mucinous Pancreatic Cysts. Am J Gastroenterol 2015; 110:909.
  45. Springer S, Wang Y, Dal Molin M, et al. A combination of molecular markers and clinical features improve the classification of pancreatic cysts. Gastroenterology 2015; 149:1501.
  46. Singhi AD, Nikiforova MN, Fasanella KE, et al. Preoperative GNAS and KRAS testing in the diagnosis of pancreatic mucinous cysts. Clin Cancer Res 2014; 20:4381.
  47. Nikiforova MN, Khalid A, Fasanella KE, et al. Integration of KRAS testing in the diagnosis of pancreatic cystic lesions: a clinical experience of 618 pancreatic cysts. Mod Pathol 2013; 26:1478.
  48. Wu J, Matthaei H, Maitra A, et al. Recurrent GNAS mutations define an unexpected pathway for pancreatic cyst development. Sci Transl Med 2011; 3:92ra66.
  49. Furukawa T, Kuboki Y, Tanji E, et al. Whole-exome sequencing uncovers frequent GNAS mutations in intraductal papillary mucinous neoplasms of the pancreas. Sci Rep 2011; 1:161.
  50. Kanda M, Sadakari Y, Borges M, et al. Mutant TP53 in duodenal samples of pancreatic juice from patients with pancreatic cancer or high-grade dysplasia. Clin Gastroenterol Hepatol 2013; 11:719.
  51. Schönleben F, Qiu W, Ciau NT, et al. PIK3CA mutations in intraductal papillary mucinous neoplasm/carcinoma of the pancreas. Clin Cancer Res 2006; 12:3851.
  52. Garcia-Carracedo D, Turk AT, Fine SA, et al. Loss of PTEN expression is associated with poor prognosis in patients with intraductal papillary mucinous neoplasms of the pancreas. Clin Cancer Res 2013; 19:6830.
  53. Frossard JL, Amouyal P, Amouyal G, et al. Performance of endosonography-guided fine needle aspiration and biopsy in the diagnosis of pancreatic cystic lesions. Am J Gastroenterol 2003; 98:1516.
  54. Jones EC, Suen KC, Grant DR, Chan NH. Fine-needle aspiration cytology of neoplastic cysts of the pancreas. Diagn Cytopathol 1987; 3:238.
  55. Centeno BA, Lewandrowski KB, Warshaw AL, et al. Cyst fluid cytologic analysis in the differential diagnosis of pancreatic cystic lesions. Am J Clin Pathol 1994; 101:483.
  56. Khalid A, Brugge W. ACG practice guidelines for the diagnosis and management of neoplastic pancreatic cysts. Am J Gastroenterol 2007; 102:2339.
  57. Cellier C, Cuillerier E, Palazzo L, et al. Intraductal papillary and mucinous tumors of the pancreas: accuracy of preoperative computed tomography, endoscopic retrograde pancreatography and endoscopic ultrasonography, and long-term outcome in a large surgical series. Gastrointest Endosc 1998; 47:42.
  58. Sugiyama M, Atomi Y, Saito M. Intraductal papillary tumors of the pancreas: evaluation with endoscopic ultrasonography. Gastrointest Endosc 1998; 48:164.
  59. Pais SA, Attasaranya S, Leblanc JK, et al. Role of endoscopic ultrasound in the diagnosis of intraductal papillary mucinous neoplasms: correlation with surgical histopathology. Clin Gastroenterol Hepatol 2007; 5:489.
  60. Tanno S, Nakano Y, Nishikawa T, et al. Natural history of branch duct intraductal papillary-mucinous neoplasms of the pancreas without mural nodules: long-term follow-up results. Gut 2008; 57:339.
  61. Kim KW, Park SH, Pyo J, et al. Imaging features to distinguish malignant and benign branch-duct type intraductal papillary mucinous neoplasms of the pancreas: a meta-analysis. Ann Surg 2014; 259:72.
  62. Grützmann R, Niedergethmann M, Pilarsky C, et al. Intraductal papillary mucinous tumors of the pancreas: biology, diagnosis, and treatment. Oncologist 2010; 15:1294.
  63. Aithal GP, Chen RY, Cunningham JT, et al. Accuracy of EUS for detection of intraductal papillary mucinous tumor of the pancreas. Gastrointest Endosc 2002; 56:701.
  64. Fasanella KE, McGrath K. Cystic lesions and intraductal neoplasms of the pancreas. Best Pract Res Clin Gastroenterol 2009; 23:35.
  65. Bardales RH, Centeno B, Mallery JS, et al. Endoscopic ultrasound-guided fine-needle aspiration cytology diagnosis of solid-pseudopapillary tumor of the pancreas: a rare neoplasm of elusive origin but characteristic cytomorphologic features. Am J Clin Pathol 2004; 121:654.
  66. Jani N, Dewitt J, Eloubeidi M, et al. Endoscopic ultrasound-guided fine-needle aspiration for diagnosis of solid pseudopapillary tumors of the pancreas: a multicenter experience. Endoscopy 2008; 40:200.
  67. Nickl NJ, Lawson JM, Cotton PB. Mucinous pancreatic tumors: ERCP findings. Gastrointest Endosc 1991; 37:133.
  68. Raijman I, Kortan P, Walden D, et al. Mucinous ductal ectasia: cholangiopancreatographic and endoscopic findings. Endoscopy 1994; 26:303.
  69. Tanaka M, Fernández-del Castillo C, Adsay V, et al. International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology 2012; 12:183.
  70. Gan SI, Thompson CC, Lauwers GY, et al. Ethanol lavage of pancreatic cystic lesions: initial pilot study. Gastrointest Endosc 2005; 61:746.
  71. Oh HC, Seo DW, Lee TY, et al. New treatment for cystic tumors of the pancreas: EUS-guided ethanol lavage with paclitaxel injection. Gastrointest Endosc 2008; 67:636.
  72. Oh HC, Seo DW, Song TJ, et al. Endoscopic ultrasonography-guided ethanol lavage with paclitaxel injection treats patients with pancreatic cysts. Gastroenterology 2011; 140:172.
  73. King JC, Ng TT, White SC, et al. Pancreatic serous cystadenocarcinoma: a case report and review of the literature. J Gastrointest Surg 2009; 13:1864.
  74. Jais B, Rebours V, Malleo G, et al. Serous cystic neoplasm of the pancreas: a multinational study of 2622 patients under the auspices of the International Association of Pancreatology and European Pancreatic Club (European Study Group on Cystic Tumors of the Pancreas). Gut 2016; 65:305.
  75. Lee SE, Jang JY, Hwang DW, et al. Clinical features and outcome of solid pseudopapillary neoplasm: differences between adults and children. Arch Surg 2008; 143:1218.
  76. Kim MJ, Choi DW, Choi SH, et al. Surgical treatment of solid pseudopapillary neoplasms of the pancreas and risk factors for malignancy. Br J Surg 2014; 101:1266.
  77. Law JK, Ahmed A, Singh VK, et al. A systematic review of solid-pseudopapillary neoplasms: are these rare lesions? Pancreas 2014; 43:331.
  78. Chen X, Zhou GW, Zhou HJ, et al. Diagnosis and treatment of solid-pseudopapillary tumors of the pancreas. Hepatobiliary Pancreat Dis Int 2005; 4:456.
  79. Alexandrescu DT, O'Boyle K, Feliz A, et al. Metastatic solid-pseudopapillary tumour of the pancreas: clinico-biological correlates and management. Clin Oncol (R Coll Radiol) 2005; 17:358.