Tools
Pancreatic Cysts
Introduction
The pancreas is an important organ in the digestive system that produces enzymes for digestion and releases important hormones into our circulation that helps with metabolism. Pancreatic cysts are often detected in patients undergoing imaging with either CT or MRI for unrelated reasons due to a combination of an aging population and the overall increased use of imaging. Pancreatic cysts are identified in 2% to 20% of patients undergoing CT or MRI; however, these rates can be lower in those with no history of pancreatitis.[1],[2],[3] On the other hand, these rates can increase to nearly one-third of patients in high-risk populations with a family history of pancreatic cancer.[4],[5]
These cysts can be either neoplastic or nonneoplastic. Neoplastic cysts include intraductal papillary mucinous neoplasms (IPMN), mucinous cystic neoplasm, solid pseudopapillary neoplasm, and cystic pancreatic neuroendocrine tumors. IPMNs can be subclassified as main duct IPMN, branch duct IPMN, and mixed IPMN. Nonneoplastic cysts include serous cystic adenoma, simple cysts, lymphoepithelial cysts, and mucinous nonneoplastic cysts. Correct diagnosis is important as management drastically changes if the cyst is neoplastic or nonneoplastic.
Moreover, surgical resection is not always the best option for all pancreatic cysts as resection can be complicated and have many risks associated with it. Depending on the cyst type, growth pattern, and symptoms, surveillance can sometimes be the best option. Moreover, with the ever-growing concern about pancreatic cysts and their risk of malignancy, new advances, such as endoscopic ultrasound, have greatly improved surveillance and proper diagnosis to guide treatment options.
Etiology
Register For Free And Read The Full Article
Search engine and full access to all medical articles- 10 free questions in your specialty
- Free CME/CE Activities
- Free daily question in your email
- Save favorite articles to your dashboard
- Emails offering discounts
Learn more about a Subscription to StatPearls Point-of-Care
Etiology
The etiology of pancreatic cysts varies widely depending on if they are non-neoplastic or neoplastic. Moreover, they are often found incidentally. As above, a history of pancreatic cancer and/or pancreatitis can increase the risk of developing pancreatic cysts. Neoplastic cysts include IPMN which includes main duct IPMN, branch duct IPMN, and mixed IPMN, mucinous cystic neoplasm, solid pseudopapillary neoplasm, and cystic pancreatic neuroendocrine tumors. Nonneoplastic cysts include serous cystic adenoma, simple cysts, lymphoepithelial cysts, and mucinous nonneoplastic cysts.
Epidemiology
The true frequency and incidence of pancreatic cysts is unknown; however, in a surgical case series of resected pancreatic cysts, the frequency was 26% branch duct IPMN, 25% main duct IPMN, 13% to 23% serous cystadenoma, 11% to 18% mucinous cystic neoplasm, 4% to 7% cystic pancreatic neuroendocrine tumor, and 2% solid pseudopapillary neoplasm.[1],[6],[7]
Pathophysiology
IPMN: The three types of IPMN are main duct, branch duct, and mixed. Main duct IPMN is characterized by segmental or diffuse dilation of the main pancreatic duct to greater than 5 mm in diameter without any other causes of obstruction. Branch duct IPMN is characterized by cysts greater than 5 mm in diameter that communicate with the main pancreatic duct without main duct dilation. Mixed IPMN has cysts that meet criteria for both main duct and branch duct IPMN.[1],[8]
Mucinous cystic neoplasms: Large, mucin-producing cysts that do not communicate with the ductal system.[1] The finding of the ovarian-type stroma is diagnostic for this lesion.[9]
Solid pseudopapillary neoplasm: Large, well-demarcated, mixed cystic and solid tumors.[1]
Cystic pancreatic neuroendocrine tumor: Smaller, more likely nonfunctional, cystic lesions that may be associated with multiple endocrine neoplasia types 1.[1]
Serous cystadenoma: Two subtypes include microcystic type serous cystadenoma and macrocystic serous cystadenoma. Microcystic type serous cystadenoma pathognomonically can be recognized by a honeycomb appearance composed of small septated cysts around a central stellate scar.[1],[10],[11]. Macrocystic serous cystadenomas are radiologically indistinguishable from other pancreatic mucinous lesions.[1],[12]
Simple cyst and lymphoepithelial cyst: Lymphoepithelial cysts have nondysplastic squamous cells with sheets of benign lymphocytes.[13]
Mucinous nonneoplastic cyst: Mucin-producing cyst without malignant potential that is differentiated from mucinous cystic neoplasm by lack of ovarian-type stroma and from IPMN by lack of ductal communication.[1],[14],[15],[16]
History and Physical
Important history would include a history of pancreatitis, a family history of pancreatic cancer, and MEN type 1. Patients are typically asymptomatic; however, they can have abdominal pain, back pain, weight loss, jaundice, steatorrhea, or a palpable mass.[17],[18]. Given the signs and symptoms are nonspecific, a detailed history of other differentials is vital to differentiate the cause. Thus, other notable history would also include alcohol and smoking intake; NSAID use; history of liver, pancreatic, peptic or biliary disease; fevers; or chills.
Evaluation
Pancreatic cysts are usually found incidentally via CT or MRI. The further evaluation depends on findings on these imaging modalities and symptomatology. Moreover, guidelines for the evaluation of pancreatic cysts continue to be modified as advanced diagnostic tests such as endoscopic ultrasound are developed.
The American Gastroenterology Association has guidelines for patients with asymptomatic, incidental pancreatic cysts.[19],[20] Incidental cysts first should be classified as either high risk or low risk for malignancy on imaging and patient presentation. High-risk features include a symptomatic patient, lymphadenopathy, main pancreatic duct diameter greater than 5 mm, and cyst characteristics that include an abrupt change in the main pancreatic duct caliber, mural nodule, enhancing solid component, thickened walls, and a cyst size of greater than 3 cm.[1] Based on these risk factors, cysts are then split into surveillance only, immediate endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA), or surgery. EUS-FNA is warranted if at least two of the following are met: size greater than 3 cm, solid component, or main pancreatic duct dilation.[1]
For symptomatic pancreatic cysts, surgical resection is the preferred treatment (ACCF/AHA class 1, level B recommendation) as it will provide relief of symptoms but also because pain is associated with a greater likelihood of premalignant or malignant pathology.[1][17][18][21][22]
High-risk pancreatic cysts that have the high-risk features above or a main pancreatic duct dilation, suspected mucinous cystic neoplasm, presumed branch duct IPMN greater than 3 cm are recommended as an ACCF/AHA class 1, level B for resection.[1]
For low-risk pancreatic cysts such as microcystic serous cystadenoma, they likely can be observed regardless of size as they have very low levels of malignant potential. However, macrocystic serous cystadenomas can be hard to distinguish from mucinous lesions on imaging and require evaluation by EUS-FNA, in which the fluid would be tested for carcinoembryonic antigen level (CEA).[1]
EUS-FNA is a procedure that is gaining ground for further characterizing lesions and prevent misdiagnosis. Cyst fluid can be analyzed for mucin, CEA, and cytology. High CEA levels are correlated with mucinous lesions. Moreover, fluid cytology can help distinguish malignant versus nonmalignant lesions.[1]
Treatment / Management
Management ultimately depends on initial imaging and symptom findings. As per the American Gastroenterological Association, initial management for asymptomatic incidental cysts can be split into three categories: surveillance, need for EUS-FNA, or surgery.[23]. Surveillance is recommended if the cyst is less than 3 cm, no solid component, and no main pancreatic duct dilation. EUS-FNA is recommended if at least two criteria are met from a size greater than 3 cm, solid component, or main pancreatic duct dilation. Surgery is recommended if there is both a solid component and main pancreatic duct dilation or EUS-FNA shows suspicious findings. If surveillance is the treatment, then an MRI should be repeated in 1 year and then every 2 years after that. If there is no change in the cyst, surveillance can be stopped after 5 years. If there is a change, then EUS-FNA should be done. If the initial option was surgical resection and the histology returns as high-grade dysplasia or cancer, then MRI should be performed every 2 years thereafter.[1]
All main duct IPMN, mixed IPMN, and mucinous cystic neoplasms should be resected. Branch duct IPMN resection depends on if the patient is symptomatic, presence of enhancing solid cyst component, main pancreatic duct diameter greater than 5mm, mural nodule, cyst fluid cytology shows suspicious or positive findings of malignancy or a change in main pancreatic duct caliber with distal pancreatic atrophy.[1]
Differential Diagnosis
Given the nonspecific physical exam findings, the differential for pancreatic cysts can be broad. One must rule out pancreatitis, liver, biliary, and peptic diseases. Differentiation can be done via proper history taking, physical exam, laboratory tests, and imaging. Since pancreatic cysts are often found incidentally on imaging, some of these differentials can be ruled out concomitantly.
Prognosis
Depending on the type of the pancreatic cyst, the risk of malignancy varies. Noninvasive diseases typically have a great prognosis.[1]
Five-year survival:
- Resected noninvasive main duct IPMN nearly 100%, however, five-year survival for malignant lesions is around 60%.[1],[24]
- Resected malignant branch duct IPMN around 70%.[1],[25]
- Noninvasive mucinous cystic neoplasm approximately 96% and invasive disease following resection approximately 75%.[1],[26],[27]
- cystic pancreatic neuroendocrine tumor approximately 77%.[1],[28],[29],[30],[31]
- limited data is available for solid pseudopapillary neoplasm due to the rarity of the condition.[1]
Deterrence and Patient Education
It is important to monitor the signs and symptoms described above. If present, seeking medical attention is advised.
Pearls and Other Issues
Differentiating pancreatic cysts can be a difficult endeavor, and EUS-FNA has provided a useful modality to determine the etiologies of these cysts.
Enhancing Healthcare Team Outcomes
Consultation with a gastroenterologist is recommended because the complexity of pancreatic cysts makes management dependent on correct diagnosis and surveillance. Moreover, advanced procedures such as EUS-FNA are required in the diagnosis of the cyst. Once a pancreatic cyst has been diagnosed, an interprofessional team will be needed to guide management. The team can consist of the primary care physician, gastroenterologist, radiologist, pathologist, and surgeon.[1]The outlook for simple pancreatic cysts is good but complex cysts may require a surgical drainage procedure, which also adds to the morbidity. Finally, it is imperative that malignancy always be considered in the differential.
References
Stark A, Donahue TR, Reber HA, Hines OJ. Pancreatic Cyst Disease: A Review. JAMA. 2016 May 3:315(17):1882-93. doi: 10.1001/jama.2016.4690. Epub [PubMed PMID: 27139061]
Zhang XM, Mitchell DG, Dohke M, Holland GA, Parker L. Pancreatic cysts: depiction on single-shot fast spin-echo MR images. Radiology. 2002 May:223(2):547-53 [PubMed PMID: 11997566]
de Jong K, Nio CY, Hermans JJ, Dijkgraaf MG, Gouma DJ, van Eijck CH, van Heel E, Klass G, Fockens P, Bruno MJ. High prevalence of pancreatic cysts detected by screening magnetic resonance imaging examinations. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association. 2010 Sep:8(9):806-11. doi: 10.1016/j.cgh.2010.05.017. Epub 2010 Jun 1 [PubMed PMID: 20621679]
Matsubara S, Tada M, Akahane M, Yagioka H, Kogure H, Sasaki T, Arizumi T, Togawa O, Nakai Y, Sasahira N, Hirano K, Tsujino T, Isayama H, Toda N, Kawabe T, Ohtomo K, Omata M. Incidental pancreatic cysts found by magnetic resonance imaging and their relationship with pancreatic cancer. Pancreas. 2012 Nov:41(8):1241-6. doi: 10.1097/MPA.0b013e31824f5970. Epub [PubMed PMID: 22699201]
Tada M, Kawabe T, Arizumi M, Togawa O, Matsubara S, Yamamoto N, Nakai Y, Sasahira N, Hirano K, Tsujino T, Tateishi K, Isayama H, Toda N, Yoshida H, Omata M. Pancreatic cancer in patients with pancreatic cystic lesions: a prospective study in 197 patients. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association. 2006 Oct:4(10):1265-70 [PubMed PMID: 16979953]
Ferrone CR, Correa-Gallego C, Warshaw AL, Brugge WR, Forcione DG, Thayer SP, Fernández-del Castillo C. Current trends in pancreatic cystic neoplasms. Archives of surgery (Chicago, Ill. : 1960). 2009 May:144(5):448-54. doi: 10.1001/archsurg.2009.36. Epub [PubMed PMID: 19451487]
Level 2 (mid-level) evidenceGaujoux S, Brennan MF, Gonen M, D'Angelica MI, DeMatteo R, Fong Y, Schattner M, DiMaio C, Janakos M, Jarnagin WR, Allen PJ. Cystic lesions of the pancreas: changes in the presentation and management of 1,424 patients at a single institution over a 15-year time period. Journal of the American College of Surgeons. 2011 Apr:212(4):590-600; discussion 600-3. doi: 10.1016/j.jamcollsurg.2011.01.016. Epub [PubMed PMID: 21463795]
Level 2 (mid-level) evidenceTanaka M, Fernández-del Castillo C, Adsay V, Chari S, Falconi M, Jang JY, Kimura W, Levy P, Pitman MB, Schmidt CM, Shimizu M, Wolfgang CL, Yamaguchi K, Yamao K, International Association of Pancreatology. International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]. 2012 May-Jun:12(3):183-97. doi: 10.1016/j.pan.2012.04.004. Epub 2012 Apr 16 [PubMed PMID: 22687371]
Level 3 (low-level) evidenceMurakami Y, Uemura K, Ohge H, Hayashidani Y, Sudo T, Sueda T. Intraductal papillary-mucinous neoplasms and mucinous cystic neoplasms of the pancreas differentiated by ovarian-type stroma. Surgery. 2006 Sep:140(3):448-53 [PubMed PMID: 16934608]
Level 2 (mid-level) evidenceGoh BK, Tan YM, Yap WM, Cheow PC, Chow PK, Chung YF, Wong WK, Ooi LL. Pancreatic serous oligocystic adenomas: clinicopathologic features and a comparison with serous microcystic adenomas and mucinous cystic neoplasms. World journal of surgery. 2006 Aug:30(8):1553-9 [PubMed PMID: 16773248]
Level 2 (mid-level) evidenceLee SE, Kwon Y, Jang JY, Kim YH, Hwang DW, Kim MA, Kim SH, Kim SW. The morphological classification of a serous cystic tumor (SCT) of the pancreas and evaluation of the preoperative diagnostic accuracy of computed tomography. Annals of surgical oncology. 2008 Aug:15(8):2089-95. doi: 10.1245/s10434-008-9959-1. Epub 2008 May 14 [PubMed PMID: 18478300]
Khurana B, Mortelé KJ, Glickman J, Silverman SG, Ros PR. Macrocystic serous adenoma of the pancreas: radiologic-pathologic correlation. AJR. American journal of roentgenology. 2003 Jul:181(1):119-23 [PubMed PMID: 12818841]
Level 2 (mid-level) evidenceKavuturu S, Sarwani NE, Ruggeiro FM, Deshaies I, Kimchi ET, Kaifi JT, Staveley-O'Carroll KF, Gusani NJ. Lymphoepithelial cysts of the pancreas. Can preoperative imaging distinguish this benign lesion from malignant or pre-malignant cystic pancreatic lesions? JOP : Journal of the pancreas. 2013 May 10:14(3):250-5. doi: 10.6092/1590-8577/1229. Epub 2013 May 10 [PubMed PMID: 23669473]
Level 3 (low-level) evidenceKosmahl M, Egawa N, Schröder S, Carneiro F, Lüttges J, Klöppel G. Mucinous nonneoplastic cyst of the pancreas: a novel nonneoplastic cystic change? Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc. 2002 Feb:15(2):154-8 [PubMed PMID: 11850544]
Keswani RN, Bentrem DJ, Hayman A, Zhu B, Lin X. Clinical characteristics and outcomes of surgically resected mucinous nonneoplastic cysts of the pancreas. Pancreas. 2012 Jul:41(5):813-5. doi: 10.1097/MPA.0b013e318243a276. Epub [PubMed PMID: 22695095]
Level 3 (low-level) evidenceNadig SN, Pedrosa I, Goldsmith JD, Callery MP, Vollmer CM. Clinical implications of mucinous nonneoplastic cysts of the pancreas. Pancreas. 2012 Apr:41(3):441-6. doi: 10.1097/MPA.0b013e318229b9b8. Epub [PubMed PMID: 22015974]
Level 2 (mid-level) evidenceFernández-del Castillo C, Targarona J, Thayer SP, Rattner DW, Brugge WR, Warshaw AL. Incidental pancreatic cysts: clinicopathologic characteristics and comparison with symptomatic patients. Archives of surgery (Chicago, Ill. : 1960). 2003 Apr:138(4):427-3; discussion 433-4 [PubMed PMID: 12686529]
Level 2 (mid-level) evidenceWalsh RM, Henderson JM, Vogt DP, Baker ME, O'malley CM Jr, Herts B, Zuccaro G Jr, Vargo JJ, Dumot JA, Conwell DL, Biscotti CV, Brown N. Prospective preoperative determination of mucinous pancreatic cystic neoplasms. Surgery. 2002 Oct:132(4):628-33; discussion 633-4 [PubMed PMID: 12407346]
Level 2 (mid-level) evidenceScheiman JM, Hwang JH, Moayyedi P. American gastroenterological association technical review on the diagnosis and management of asymptomatic neoplastic pancreatic cysts. Gastroenterology. 2015 Apr:148(4):824-48.e22. doi: 10.1053/j.gastro.2015.01.014. Epub [PubMed PMID: 25805376]
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 Apr:148(4):819-22; quize12-3. doi: 10.1053/j.gastro.2015.01.015. Epub [PubMed PMID: 25805375]
Spinelli KS, Fromwiller TE, Daniel RA, Kiely JM, Nakeeb A, Komorowski RA, Wilson SD, Pitt HA. Cystic pancreatic neoplasms: observe or operate. Annals of surgery. 2004 May:239(5):651-7; discussion 657-9 [PubMed PMID: 15082969]
Level 2 (mid-level) evidenceChaudhari VV, Raman SS, Vuong NL, Zimmerman P, Farrell J, Reber H, Sayre J, Lu DS. Pancreatic cystic lesions: discrimination accuracy based on clinical data and high-resolution computed tomographic features. Journal of computer assisted tomography. 2008 Sep-Oct:32(5):757-63. doi: 10.1097/RCT.0b013e318157b100. Epub [PubMed PMID: 18830106]
Level 2 (mid-level) evidenceKawakubo K, Tada M, Isayama H, Sasahira N, Nakai Y, Takahara N, Uchino R, Hamada T, Miyabayashi K, Yamamoto K, Mizuno S, Mohri D, Kogure H, Sasaki T, Yamamoto N, Hirano K, Ijichi H, Tateishi K, Koike K. Disease-specific mortality among patients with intraductal papillary mucinous neoplasm of the pancreas. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association. 2014 Mar:12(3):486-91. doi: 10.1016/j.cgh.2013.06.032. Epub 2013 Jul 25 [PubMed PMID: 23892276]
Salvia R, Fernández-del Castillo C, Bassi C, Thayer SP, Falconi M, Mantovani W, Pederzoli P, Warshaw AL. Main-duct intraductal papillary mucinous neoplasms of the pancreas: clinical predictors of malignancy and long-term survival following resection. Annals of surgery. 2004 May:239(5):678-85; discussion 685-7 [PubMed PMID: 15082972]
Jang JY, Kim SW, Lee SE, Yang SH, Lee KU, Lee YJ, Kim SC, Han DJ, Choi DW, Choi SH, Heo JS, Cho BH, Yu HC, Yoon DS, Lee WJ, Lee HE, Kang GH, Lee JM. Treatment guidelines for branch duct type intraductal papillary mucinous neoplasms of the pancreas: when can we operate or observe? Annals of surgical oncology. 2008 Jan:15(1):199-205 [PubMed PMID: 17909912]
Level 2 (mid-level) evidenceYamao K, Yanagisawa A, Takahashi K, Kimura W, Doi R, Fukushima N, Ohike N, Shimizu M, Hatori T, Nobukawa B, Hifumi M, Kobayashi Y, Tobita K, Tanno S, Sugiyama M, Miyasaka Y, Nakagohri T, Yamaguchi T, Hanada K, Abe H, Tada M, Fujita N, Tanaka M. Clinicopathological features and prognosis of mucinous cystic neoplasm with ovarian-type stroma: a multi-institutional study of the Japan pancreas society. Pancreas. 2011 Jan:40(1):67-71. doi: 10.1097/MPA.0b013e3181f749d3. Epub [PubMed PMID: 20924309]
Level 2 (mid-level) evidencePark JW, Jang JY, Kang MJ, Kwon W, Chang YR, Kim SW. Mucinous cystic neoplasm of the pancreas: is surgical resection recommended for all surgically fit patients? Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]. 2014 Mar-Apr:14(2):131-6. doi: 10.1016/j.pan.2013.12.006. Epub 2014 Jan 8 [PubMed PMID: 24650968]
Level 2 (mid-level) evidenceBordeianou L, Vagefi PA, Sahani D, Deshpande V, Rakhlin E, Warshaw AL, Fernández-del Castillo C. Cystic pancreatic endocrine neoplasms: a distinct tumor type? Journal of the American College of Surgeons. 2008 Jun:206(6):1154-8. doi: 10.1016/j.jamcollsurg.2007.12.040. Epub 2008 Apr 14 [PubMed PMID: 18501813]
Level 2 (mid-level) evidenceCharfi S, Marcy M, Bories E, Pesanti C, Caillol F, Giovannini M, Viret F, Robert Delpero J, Xerri L, Monges G. Cystic pancreatic endocrine tumors: an endoscopic ultrasound-guided fine-needle aspiration biopsy study with histologic correlation. Cancer. 2009 Jun 25:117(3):203-10. doi: 10.1002/cncy.20024. Epub [PubMed PMID: 19365841]
Level 3 (low-level) evidenceGaujoux S, Tang L, Klimstra D, Gonen M, Brennan MF, D'Angelica M, DeMatteo R, Fong Y, Jarnagin W, Allen PJ. The outcome of resected cystic pancreatic endocrine neoplasms: a case-matched analysis. Surgery. 2012 Apr:151(4):518-25. doi: 10.1016/j.surg.2011.09.037. Epub 2011 Nov 16 [PubMed PMID: 22088817]
Level 2 (mid-level) evidenceKawamoto S, Johnson PT, Shi C, Singhi AD, Hruban RH, Wolfgang CL, Edil BH, Fishman EK. Pancreatic neuroendocrine tumor with cystlike changes: evaluation with MDCT. AJR. American journal of roentgenology. 2013 Mar:200(3):W283-90. doi: 10.2214/AJR.12.8941. Epub [PubMed PMID: 23436873]