Background: With the use of modern cross-sectional abdominal imaging modalities, an increasing number of cystic pancreatic lesions are identified incidentally. Although there is no pathological diagnosis available in most cases, it is believed that the majority of these lesions display small branch-duct intraductal papillary mucinous neoplasms (BD-IPMNs) of the pancreas. Even though a number of large clinical series have been published, many uncertainties remain with regard to this entity of mucinous cystic neoplasms. Methods: Systematic literature review. Results: Main-duct (MD) and mixed-type IPMNs harbor a high risk of malignant transformation. It is conceivable that most IPMNs with involvement of the main duct tend to progress to invasive carcinoma over time. Thus, formal oncologic resection is the treatment of choice in surgically fit patients. In contrast, the data regarding BD-IPMN remain equivocal, resulting in conflicting concepts. To date, it is not clear whether and which BD-IPMNs progress to carcinoma and how long this progression takes. Conclusion: While patients with MD-IPMNs should undergo surgical resection if comorbidities and life expectancy permit this, the management of small BD-IPMNs remains controversial. Population-based studies with long-term follow-up are needed to define which cohort of patients can be observed safely without immediate resection.

1.
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;8:806-811.
2.
Laffan TA, Horton KM, Klein AP, et al: Prevalence of unsuspected pancreatic cysts on MDCT. AJR Am J Roentgenol 2008;191:802-807.
3.
Fernandez-del Castillo C, Adsay NV: Intraductal papillary mucinous neoplasms of the pancreas. Gastroenterology 2010;139:708-713, 713.e701-702.
4.
Farrell JJ, Garber J, Sahani D, Brugge WR: EUS findings in patients with autoimmune pancreatitis. Gastrointest Endosc 2004;60:927-936.
5.
Longnecker DS: Intraductal papillary-mucinous tumors of the pancreas. Arch Pathol Lab Med 1995;119:197-198.
6.
Sohn TA, Yeo CJ, Cameron JL, et al: Intraductal papillary mucinous neoplasms of the pancreas: an updated experience. Ann Surg 2004;239:788-797; discussion 797-789.
7.
Tanno S, Nakano Y, Koizumi K, et al: Pancreatic ductal adenocarcinomas in long-term follow-up patients with branch duct intraductal papillary mucinous neoplasms. Pancreas 2010;39:36-40.
8.
Salvia R, Crippa S, Partelli S, et al: Differences between main-duct and branch-duct intraductal papillary mucinous neoplasms of the pancreas. World J Gastrointest Surg 2010;2:342-346.
9.
Spinelli KS, Fromwiller TE, Daniel RA, et al: Cystic pancreatic neoplasms: observe or operate. Ann Surg 2004;239:651-657; discussion 657-659.
10.
Tanaka M, Fernandez-del Castillo C, Adsay V, et al: International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology 2012;12:183-197.
11.
Schmidt CM, White PB, Waters JA, et al: Intraductal papillary mucinous neoplasms: predictors of malignant and invasive pathology. Ann Surg 2007;246:644-651; discussion 651-654.
12.
Jang JY, Kim SW, Lee SE, et al: Treatment guidelines for branch duct type intraductal papillary mucinous neoplasms of the pancreas: when can we operate or observe? Ann Surg Oncol 2008;15:199-205.
13.
Fritz S, Klauss M, Bergmann F, et al: Small (Sendai negative) branch-duct IPMNs: not harmless. Ann Surg 2012;256:313-320.
14.
Wong J, Weber J, Centeno BA, et al: High-grade dysplasia and adenocarcinoma are frequent in side-branch intraductal papillary mucinous neoplasm measuring less than 3 cm on endoscopic ultrasound. J Gastrointest Surg 2013;17:78-84; discussion 84-85.
15.
Conlon KC: Intraductal papillary mucinous tumors of the pancreas. J Clin Oncol 2005;23:4518-4523.
16.
Salvia R, Fernandez-del Castillo C, Bassi C, et al: Main-duct intraductal papillary mucinous neoplasms of the pancreas: clinical predictors of malignancy and long-term survival following resection. Ann Surg 2004;239:678-685; discussion 685-687.
17.
Ingkakul T, Warshaw AL, Fernandez-Del Castillo C: Epidemiology of intraductal papillary mucinous neoplasms of the pancreas: sex differences between 3 geographic regions. Pancreas 2011;40:779-780.
18.
Sahora K, Mino-Kenudson M, Brugge W, et al: Branch duct intraductal papillary mucinous neoplasms: does cyst size change the tip of the scale? A critical analysis of the revised international consensus guidelines in a large single-institutional series. Ann Surg 2013;258:466-475.
19.
Fernandez-del Castillo C, Targarona J, Thayer SP, Rattner DW, Brugge WR, Warshaw AL: Incidental pancreatic cysts: clinicopathologic characteristics and comparison with symptomatic patients. Arch Surg 2003;138:427-433; discussion 433-434.
20.
Salvia R, Crippa S, Falconi M, et al: Branch-duct intraductal papillary mucinous neoplasms of the pancreas: to operate or not to operate? Gut 2007;56:1086-1090.
21.
Ferrone CR, Correa-Gallego C, Warshaw AL, et al: Current trends in pancreatic cystic neoplasms. Arch Surg 2009;144:448-454.
22.
Sohn TA, Yeo CJ, Cameron JL, Iacobuzio-Donahue CA, Hruban RH, Lillemoe KD: Intraductal papillary mucinous neoplasms of the pancreas: an increasingly recognized clinicopathologic entity. Ann Surg 2001;234:313-321; discussion 321-322.
23.
Rodriguez JR, Salvia R, Crippa S, et al: Branch-duct intraductal papillary mucinous neoplasms: observations in 145 patients who underwent resection. Gastroenterology 2007;133:72-79; quiz 309-310.
24.
Sato N, Rosty C, Jansen M, et al: STK11/LKB1 Peutz-Jeghers gene inactivation in intraductal papillary-mucinous neoplasms of the pancreas. Am J Pathol 2001;159:2017-2022.
25.
Maire F, Hammel P, Terris B, et al: Intraductal papillary and mucinous pancreatic tumour: a new extracolonic tumour in familial adenomatous polyposis. Gut 2002;51:446-449.
26.
Abe T, Fukushima N, Brune K, et al: Genome-wide allelotypes of familial pancreatic adenocarcinomas and familial and sporadic intraductal papillary mucinous neoplasms. Clin Cancer Res 2007;13:6019-6025.
27.
Ingkakul T, Sadakari Y, Ienaga J, Satoh N, Takahata S, Tanaka M: Predictors of the presence of concomitant invasive ductal carcinoma in intraductal papillary mucinous neoplasm of the pancreas. Ann Surg 2010;251:70-75.
28.
Yamaguchi K, Yokohata K, Noshiro H, Chijiiwa K, Tanaka M: Mucinous cystic neoplasm of the pancreas or intraductal papillary-mucinous tumour of the pancreas. Eur J Surg 2000;166:141-148.
29.
Reid-Lombardo KM, Mathis KL, Wood CM, Harmsen WS, Sarr MG: Frequency of extrapancreatic neoplasms in intraductal papillary mucinous neoplasm of the pancreas: implications for management. Ann Surg 2010;251:64-69.
30.
Hruben RH, Pitman MB, Klimstra DS: Intraductal neoplasms; in Hruben RH, Pitman MB, Klimstra DS (eds): AFIP Atlas of Tumor Pathology, Series 4: Tumors of the Pancreas. Washington, DC, American Registry of Pathology, 2007, pp 75-110.
31.
Pelaez-Luna M, Chari ST, Smyrk TC, et al: Do consensus indications for resection in branch duct intraductal papillary mucinous neoplasm predict malignancy? A study of 147 patients. Am J Gastroenterol 2007;102:1759-1764.
32.
Kamisawa T, Fujiwara T, Tu Y, et al: Long-term follow-up of intraductal papillary adenoma of the pancreas. J Gastroenterol 2002;37:868-873.
33.
Hruban RH, Adsay NV: Molecular classification of neoplasms of the pancreas. Hum Pathol 2009;40:612-623.
34.
Adsay NV, Merati K, Basturk O, et al: 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 2004;28:839-848.
35.
Wada K, Kozarek RA, Traverso LW: Outcomes following resection of invasive and noninvasive intraductal papillary mucinous neoplasms of the pancreas. Am J Surg 2005;189:632-636; discussion 637.
36.
Mino-Kenudson M, Fernandez-Del Castillo C, Baba Y, et al: Prognosis of invasive intraductal papillary mucinous neoplasm depends on histological and precursor epithelial subtypes. Gut 2011;60:1712-1720.
37.
Crippa S, Fernandez-Del Castillo C, Salvia R, et al: Mucin-producing neoplasms of the pancreas: an analysis of distinguishing clinical and epidemiologic characteristics. Clin Gastroenterol Hepatol 2010;8:213-219.
38.
Del Chiaro M, Verbeke C, Salvia R, et al: European experts consensus statement on cystic tumours of the pancreas. Dig Liver Dis 2013;45:703-711.
39.
Farrell JJ, Fernandez-del Castillo C: Pancreatic cystic neoplasms: management and unanswered questions. Gastroenterology 2013;144:1303-1315.
40.
Chiu SS, Lim JH, Lee WJ, et al: Intraductal papillary mucinous tumour of the pancreas: differentiation of malignancy and benignancy by CT. Clin Radiol 2006;61:776-783.
41.
Tanaka M, Chari S, Adsay V, et al: International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas. Pancreatology 2006;6:17-32.
42.
Ohno E, Hirooka Y, Itoh A, et al: Intraductal papillary mucinous neoplasms of the pancreas: differentiation of malignant and benign tumors by endoscopic ultrasound findings of mural nodules. Ann Surg 2009;249:628-634.
43.
Cone MM, Rea JD, Diggs BS, Douthit MA, Billingsley KG, Sheppard BC: Predicting malignant intraductal papillary mucinous neoplasm: a single-center review. Am J Surg 2011;201:575-579.
44.
Gerke H, Jaffe TA, Mitchell RM, et al: Endoscopic ultrasound and computer tomography are inaccurate methods of classifying cystic pancreatic lesions. Dig Liver Dis 2006;38:39-44.
45.
Walsh RM, Vogt DP, Henderson JM, et al: Management of suspected pancreatic cystic neoplasms based on cyst size. Surgery 2008;144:677-684; discussion 684-685.
46.
Pais SA, Attasaranya S, Leblanc JK, Sherman S, Schmidt CM, DeWitt J: Role of endoscopic ultrasound in the diagnosis of intraductal papillary mucinous neoplasms: correlation with surgical histopathology. Clin Gastroenterol Hepatol 2007;5:489-495.
47.
Fritz S, Hackert T, Hinz U, Hartwig W, Buchler MW, Werner J: Role of serum carbohydrate antigen 19-9 and carcinoembryonic antigen in distinguishing between benign and invasive intraductal papillary mucinous neoplasm of the pancreas. Br J Surg 2011;98:104-110.
48.
Salvia R, Malleo G, Marchegiani G, et al: Pancreatic resections for cystic neoplasms: from the surgeon's presumption to the pathologist's reality. Surgery 2012;152:S135-142.
49.
Tang RS, Weinberg B, Dawson DW, et al: Evaluation of the guidelines for management of pancreatic branch-duct intraductal papillary mucinous neoplasm. Clin Gastroenterol Hepatol 2008;6:815-819; quiz 719.
50.
Jang JY, Park T, Lee S, et al: Validation of international consensus guidelines for the resection of branch duct-type intraductal papillary mucinous neoplasms. Br J Surg 2014;101:686-692.
51.
Kobayashi G, Fujita N, Maguchi H, et al: Natural history of branch duct intraductal papillary mucinous neoplasm with mural nodules: a Japan Pancreas Society multicenter study. Pancreas 2014;43:532-538.
52.
Sahora K, Castillo CF, Dong F, et al: Not all mixed-type intraductal papillary mucinous neoplasms behave like main-duct lesions: implications of minimal involvement of the main pancreatic duct. Surgery 2014;156:611-621.
53.
Roch AM, Ceppa EP, Al-Haddad MA, et al: The natural history of main duct-involved, mixed-type intraductal papillary mucinous neoplasm: parameters predictive of progression. Ann Surg 2014;260:680-690.
54.
Izawa T, Obara T, Tanno S, Mizukami Y, Yanagawa N, Kohgo Y: Clonality and field cancerization in intraductal papillary-mucinous tumors of the pancreas. Cancer 2001;92:1807-1817.
55.
Werner J, Fritz S, Buchler MW: Intraductal papillary mucinous neoplasms of the pancreas - a surgical disease. Nat Rev Gastroenterol Hepatol 2012;9:253-259.
56.
Weinberg BM, Spiegel BM, Tomlinson JS, Farrell JJ: Asymptomatic pancreatic cystic neoplasms: maximizing survival and quality of life using Markov-based clinical nomograms. Gastroenterology 2010;138:531-540.
57.
Sawai Y, Yamao K, Bhatia V, et al: Development of pancreatic cancers during long-term follow-up of side-branch intraductal papillary mucinous neoplasms. Endoscopy 2010;42:1077-1084.
58.
Barron MR, Roch AM, Waters JA, et al: Does preoperative cross-sectional imaging accurately predict main duct involvement in intraductal papillary mucinous neoplasm? J Gastrointest Surg 2014;18:447-455; discussion 5455-5456.
59.
Goh BK, Tan DM, Ho MM, Lim TK, Chung AY, Ooi LL: Utility of the Sendai consensus guidelines for branch-duct intraductal papillary mucinous neoplasms: a systematic review. J Gastrointest Surg 2014;18:1350-1357.
60.
Lee CJ, Scheiman J, Anderson MA, et al: Risk of malignancy in resected cystic tumors of the pancreas ≤3 cm in size: is it safe to observe asymptomatic patients? A multi-institutional report. J Gastrointest Surg 2008;12:234-242.
61.
Hackert T, Hinz U, Fritz S, et al: Enucleation in pancreatic surgery: indications, technique, and outcome compared to standard pancreatic resections. Langenbecks Arch Surg 2011;396:1197-1203.
62.
Poultsides GA, Reddy S, Cameron JL, et al: Histopathologic basis for the favorable survival after resection of intraductal papillary mucinous neoplasm-associated invasive adenocarcinoma of the pancreas. Ann Surg 2010;251:470-476.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.