Mucinous Cystic Neoplasms

  • Lorene Yoxtheimer
  • Abha GoyalEmail author
Part of the Essentials in Cytopathology book series (EICP, volume 28)


Mucinous cystic neoplasms (MCNs) are uncommon neoplasms of the pancreas that are characterized by a distinctive-type ovarian stroma. They tend to occur in the distal pancreas in middle-aged women. The majority of MCNs are slow growing with the prevalence of invasive carcinoma ranging from 3.9% to 34.4%. The preoperative diagnosis of MCNs is based on a multimodal approach comprising clinical features, tumor markers, imaging findings, endoscopic ultrasound (EUS) with fine needle aspiration, and cyst fluid analysis. As per the 2012 Fukuoka consensus guidelines, surgical resection is recommended for all surgically fit patients with MCNs. Although surgical resection has been the traditional management for MCNs, conservative management options are being increasingly explored. This chapter will discuss various aspects of MCNs including the clinical features, imaging findings, pathologic features, molecular genetics, and treatment and prognosis.


Mucinous cystic neoplasm Pancreatic cyst Neoplastic mucinous cyst Endoscopic ultrasound 


  1. 1.
    Fukushima N, Zamboni G. Mucinous cystic neoplasms of the pancreas: update on the surgical pathology and molecular genetics. Semin Diagn Pathol. 2014;31:467–74. Scholar
  2. 2.
    Park 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. 2014;14:131–6. Scholar
  3. 3.
    Zamboni G, Scarpa A, Bogina G, Iacono C, Bassi C, Talamini G, et al. Mucinous cystic tumors of the pancreas: clinicopathological features, prognosis, and relationship to other mucinous cystic tumors. Am J Surg Pathol. 1999;23:410–22.CrossRefGoogle Scholar
  4. 4.
    Ishida K, Sasano H, Moriya T, Takahashi Y, Sugimoto R, Mue Y, et al. Immunohistochemical analysis of steroidogenic enzymes in ovarian-type stroma of pancreatic mucinous cystic neoplasms: comparative study of subepithelial stromal cells in intraductal papillary mucinous neoplasms of the pancreas. Pathol Int. 2016;66:281–7. Scholar
  5. 5.
    Thompson LD, Becker RC, Przygodzki RM, Adair CF, Heffess CS. Mucinous cystic neoplasm (mucinous cystadenocarcinoma of low-grade malignant potential) of the pancreas: a clinicopathologic study of 130 cases. Am J Surg Pathol. 1999;23:1–16.CrossRefGoogle Scholar
  6. 6.
    Kim SY, Lee JM, Kim SH, Shin KS, Kim YJ, An SK, et al. Macrocystic neoplasms of the pancreas: CT differentiation of serous oligocystic adenoma from mucinous cystadenoma and intraductal papillary mucinous tumor. AJR Am J Roentgenol. 2006;187:1192–8.CrossRefGoogle Scholar
  7. 7.
    Cohen-Scali F, Vilgrain V, Brancatelli G, Hammel P, Vullierme MP, Sauvanet A, et al. Discrimination of unilocular macrocystic serous cystadenoma from pancreatic pseudocyst and mucinous cystadenoma with CT: initial observations. Radiology. 2003;228:727–33.CrossRefGoogle Scholar
  8. 8.
    Scourtas A, Dudley JC, Brugge WR, Kadayifci A, Mino-Kenudson M, Pitman MB. Preoperative characteristics and cytological features of 136 histologically confirmed pancreatic mucinous cystic neoplasms. Cancer Cytopathol. 2017;125:169–77. Scholar
  9. 9.
    Procacci C, Carbognin G, Accordini S, Biasiutti C, Guarise A, Lombardo F, et al. CT features of malignant mucinous cystic tumors of the pancreas. Eur Radiol. 2001;11:1626–30.CrossRefGoogle Scholar
  10. 10.
    Garces-Descovich A, Beker K, Castillo-Angeles M, Brook A, Resnick E, Shinagare S, et al. Mucinous cystic neoplasms of the pancreas: high-resolution cross-sectional imaging features with clinico-pathologic correlation. Abdom Radiol (NY). 2018;43:1413–22.–017–1326-x.CrossRefGoogle Scholar
  11. 11.
    Baker ML, Seeley ES, Pai R, Suriawinata AA, Mino-Kenudson M, Zamboni G, et al. Invasive mucinous cystic neoplasms of the pancreas. Exp Mol Pathol. 2012;93:345–9. Scholar
  12. 12.
    Albores-Saavedra J, Manivel C, Dorantes-Heredia R, Chablé-Montero F, Godoy-Valdés C, Chan-Nuñez C, et al. Nonmucinous cystadenomas of the pancreas with pancreatobiliary phenotype and ovarian-like stroma. Am J Clin Pathol. 2013;139:599–604. Scholar
  13. 13.
    Zamboni G, Fukushima N, Hruban RH, Klöppel G. Mucinous cystic neoplasms of the pancreas. In: Bosman FT, Carneiro F, Hruban RH, Theise ND, editors. WHO classification of tumors of the diagestive system. 4th ed. Lyon: IARC; 2010. p. 300–3.Google Scholar
  14. 14.
    Lüttges J, Feyerabend B, Buchelt T, Pacena M, Klöppel G. The mucin profile of noninvasive and invasive mucinous cystic neoplasms of the pancreas. Am J Surg Pathol. 2002;26:466–71.CrossRefGoogle Scholar
  15. 15.
    Pitman MB, Centeno BA, Ali SZ, Genevay M, Stelow E, Mino-Kenudson M, et al. Standardized terminology and nomenclature for pancreatobiliary cytology: the Papanicolaou Society of Cytopathology guidelines. Diagn Cytopathol. 2014;42:338–50. Scholar
  16. 16.
    Pitman MB, Layfield LJ. Guidelines for pancreaticobiliary cytology from the Papanicolaou Society of Cytopathology: a review. Cancer Cytopathol. 2014;122:399–411. Scholar
  17. 17.
    Iacobuzio-Donahue CA, Wilentz RE, Argani P, Yeo CJ, Cameron JL, Kern SE, et al. 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. 2000;24:1544–8.CrossRefGoogle Scholar
  18. 18.
    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.–014–3254–8.CrossRefPubMedGoogle Scholar
  19. 19.
    Wu J, Jiao Y, Dal Molin M, Maitra A, de Wilde RF, Wood LD, et al. Whole-exome sequencing of neoplastic cysts of the pancreas reveals recurrent mutations in components of ubiquitin-dependent pathways. Proc Natl Acad Sci U S A. 2011;108:21188–93. Scholar
  20. 20.
    Wood LD, Hruban HR. Genomic landscapes of pancreatic neoplasia. J Pathol Transl Med. 2015;49:13–22. Scholar
  21. 21.
    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.–0432.CCR-14–0513.CrossRefPubMedGoogle Scholar
  22. 22.
    Garcia-Carracedo D, Chen ZM, Qiu W, Huang AS, Tang SM, Hruban RH, et al. PIK3CA mutations in mucinous cystic neoplasms of the pancreas. Pancreas. 2014;43:245–9. Scholar
  23. 23.
    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. Scholar
  24. 24.
    Crippa S, Salvia R, Warshaw AL, Domínguez I, Bassi C, Falconi M, et al. Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients. Ann Surg. 2008;247:571–9. Scholar
  25. 25.
    European Study Group on Cystic Tumours of the Pancreas. European evidence-based guidelines on pancreatic cystic neoplasms. Gut. 2018;67:789–804.–316027.CrossRefGoogle Scholar
  26. 26.
    Wilentz RE, Albores-Saavedra J, Zahurak M, Talamini MA, Yeo CJ, Cameron JL. Pathologic examination accurately predicts prognosis in mucinous cystic neoplasms of the pancreas. Am J Surg Pathol. 1999;23:1320–7.CrossRefGoogle Scholar
  27. 27.
    Xourafas D, Tavakkoli A, Clancy TE, Ashley SW. Noninvasive intraductal papillary mucinous neoplasms and mucinous cystic neoplasms: recurrence rates and postoperative imaging follow-up. Surgery. 2015;157:473–83. Scholar
  28. 28.
    Suzuki Y, Atomi Y, Sugiyama M, Isaji S, Inui K, Kimura W, et al. Cystic neoplasm of the pancreas: a Japanese multiinstitutional study of intraductal papillary mucinous tumor and mucinous cystic tumor. Pancreas. 2004;28(3):241–6.CrossRefGoogle Scholar
  29. 29.
    Testini M, Gurrado A, Lissidini G, Venezia P, Greco L, Piccinni G. Management of mucinous cystic neoplasms of the pancreas. World J Gastroenterol. 2010;16:5682–92.CrossRefGoogle Scholar
  30. 30.
    Lewis GH, Wang H, Bellizzi AM, Klein AP, Askin FB, Schwartz LE, et al. Prognosis of minimally invasive carcinoma arising in mucinous cystic neoplasms of the pancreas. Am J Surg Pathol. 2013;37:601–5. Scholar
  31. 31.
    Hui L, Rashid A, Foo WC, Katz MH, Chatterjee D, Wang H, et al. Significance of T1a and T1b carcinoma arising in mucinous cystic neoplasm of pancreas. Am J Surg Pathol. 2018;42:578–86. Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Pathology and Laboratory MedicineWeill-Cornell Medicine, New York Presbyterian HosptialNew YorkUSA

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