Skip to main content
  • 1897 Accesses

Abstract

Mucinous cystic neoplasm (MCN) of the pancreas is one of the three most common primary cyst-forming epithelial neoplasms of the pancreas including intraductal papillary mucinous neoplasm (IPMN), serous cystic neoplasm (SCN) and MCN. From another point of view, MCN is one of the three precursors of invasive adenocarcinoma of the pancreas including pancreatic intraepithelial neoplasia (PanIN), IPMN and MCN. MCNs occur almost exclusively in the distal pancreas of middle-aged women. Grossly, MCNs typically show a “cyst-in-cyst” pattern of growth, and are well encapsulated by a thick fibrous wall. In histology, MCNs are composed of mucinous neoplastic epithelial cells and subepithelial cellular stroma called as “ovarian-type” stroma. The epithelium is dysplastic and the grade can be divided into low- and high-grade, and some MCNs have an associated invasive carcinoma. MCNs harbor several characteristic genetic and epigenetic alterations, some of which are shared with conventional invasive pancreatic ductal adenocarcinoma. Several studies suggest steroidogenesis in the “ovarian-type” stroma. A 5-year survival rate of non-invasive MCN is 100%, and MCN with T1a and T1b carcinoma also had an excellent prognosis. However, in one study, MCN with invasive carcinoma show aggressive clinical course; a 3-year and 5-year survival rate are 44% and 26%, respectively. In European guidelines, MCNs <40 mm are treated conservatively when other risk factors are absent. In international and American guidelines, on the other hand, an MCN of any size is an absolute indication for resection. Better knowledge of the pathology and molecular alterations could help in the management of patients with MCN.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover 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. Hruban RH, Pitman MB, Klimstra DS. Tumors of the Pancreas (AFIP Atlas of Tumor Pathology; 4th Series Fascicle 6) American Registry of Pathology; 2007.

    Google Scholar 

  2. Gill AJ, Klimstra DS, Lam AK, Washington MK (eds). Tumours of the pancreas. In WHO classification of tumours – 5th edition – Digestive System, 294–373, IARC Press, Lyons, 2019.

    Google Scholar 

  3. Compagno J, Oertel JE. Microcystic adenomas of the pancreas (glycogen-rich cystadenomas): a clinicopathologic study of 34 cases. Am J Clin Pathol. 1978;69:289–98.

    Article  CAS  Google Scholar 

  4. Ohashi K, Murakami Y, Maruyama M. Four cases of a mucous secreting pancreatic cancer [in Japanese]. Prog Dig Endosc. 1982;20:348–51.

    Google Scholar 

  5. 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;23:410–22.

    Article  CAS  Google Scholar 

  6. 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.

    Article  CAS  Google Scholar 

  7. Ohtsuka T, Nakamura M, Hijioka S, et al. Prediction of the probability of malignancy in mucinous cystic neoplasm of the pancreas with ovarian-type stroma: a nationwide multicenter study in Japan. Pancreas. 2020;49(2):181–6.

    Article  Google Scholar 

  8. Crippa S, Salvia R, Warshaw AL, et al. Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients. Ann Surg. 2008;247(4):571–9.

    Article  Google Scholar 

  9. Yamao K, Yanagisawa A, Takahashi K, et al. Clinicopathological features and prognosis of mucinous cystic neoplasm with ovarian-type stroma: a multi-institutional study of the Japan pancreas society. Pancreas. 2011;40:67–71.

    Article  CAS  Google Scholar 

  10. Griffin JF, Page AJ, Samaha GJ, et al. Patients with a resected pancreatic mucinous cystic neoplasm have a better prognosis than patients with an intraductal papillary mucinous neoplasm: a large single institution series. Pancreatology. 2017;17(3):490–6.

    Article  Google Scholar 

  11. Hui L, Rashid A, Foo WC, et al. Significance of T1a and T1b carcinoma arising in mucinous cystic neoplasm of pancreas. Am J Surg Pathol. 2018;42(5):578–86.

    Article  Google Scholar 

  12. Fukushima N, Mukai K, Kanai Y, et al. Intraductal papillary tumors and mucinous cystic tumors of the pancreas. Clinicopathologic study of 38 cases. Hum Pathol. 1997;28:1010–7.

    Article  CAS  Google Scholar 

  13. Fukushima N, Sato N, Yeo CJ, Cameron JL, Hruban RH, Goggins M. Characterization of mucinous cystic neoplasms (MCNs) of the pancreas using oligonucleotide-microarrays. Oncogene. 2004;23:9042–51.

    Article  CAS  Google Scholar 

  14. Baker ML, Seeley ES, Pai R, et al. Invasive mucinous cystic neoplasms of the pancreas. Exp Mol Pathol. 2012;93:345–9.

    Article  CAS  Google Scholar 

  15. Albores-Saavedra J, Manivel C, Dorantes-Heredia R, et al. Nonmucinouscystadenomas of the pancreas with pancreatobiliary phenotype and ovarian-like stroma. Am J Clin Pathol. 2013;139:599–604.

    Article  CAS  Google Scholar 

  16. Brierley J, Gospodarowicz MK, Wittekind C. TNM classification of malignant tumours. 8th ed. Hoboken, New Jersey: Wiley-Blackwell; 2017. p. 93–5.

    Google Scholar 

  17. Hruban RH, Adsay NV, Esposito I, et al. Pancreatic ductal adenocarcinoma. In WHO classification of tumours – 5th edition – Digestive System (Eds: the WHO classification of tumours editorial board), 322–332, IARC Press, Lyons, 2019.

    Google Scholar 

  18. Wu J, Jiao Y, Dal Molin M, et al. Whole-exome sequencing of neoplastic cysts of the pancreas reveals recurrent mutations in components of ubiquitin-dependent pathways. Proc Natl Acad Sci USA. 2011;108(52):21188–93.

    Article  CAS  Google Scholar 

  19. Conner JR, Mariño-Enríquez A, Mino-Kenudson M, et al. Characterization of low- and high-grade pancreatic mucinous cystic neoplasms reveals recurrent KRAS alterations in “high-risk” lesions. Pancreas. 2017;46(5):665–71.

    Article  CAS  Google Scholar 

  20. Noë M, Niknafs N, Fischer CG, et al. Genomic characterization of malignant progression in neoplastic pancreatic cysts. Nat Commun. 2020;14;11(1):4085.

    Google Scholar 

  21. Garcia-Carracedo D, Chen ZM, Qiu W, et al. PIK3CA mutations in mucinous cystic neoplasms of the pancreas. Pancreas. 2014;43(2):245–9.

    Article  CAS  Google Scholar 

  22. 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.

    Article  Google Scholar 

  23. Dal Molin M, Matthaei H, Wu J, et al. Clinicopathological correlates of activating GNAS mutations in intraductal papillary mucinous neoplasm (IPMN) of the pancreas. Ann Surg Oncol. 2013;20(12):3802–8.

    Article  Google Scholar 

  24. Ishiguro H, Kato K, Kishimoto T, et al. Expression of steroidogenic enzymes by luteinizing cells in the ovarian-type stroma of a mucin-producing cystic tumour of the pancreas. Histopathology. 2003;43:94–100.

    Article  Google Scholar 

  25. Kumata H, Murakami K, Ishida K, et al. Steroidogenesis in ovarian-like mesenchymal stroma of hepatic and pancreatic mucinous cystic neoplasms. Hepatol Res. 2018;48(12):989–99.

    Article  Google Scholar 

  26. Revoredo F, de Vinatea J, Reaño G, et al. Mucinous cystic neoplasms of the pancreas associated with pregnancy: two case reports. Medicine (Baltimore). 2020;31;99(31):e21471.

    Google Scholar 

  27. Jang KT, Park SM, Basturk O, et al. Clinicopathologic characteristics of 29 invasive carcinomas arising in 178 pancreatic mucinous cystic neoplasms with ovarian-type stroma: implications for management and prognosis. Am J Surg Pathol. 2015;39(2):179–87.

    Article  Google Scholar 

  28. 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(3):183–97.

    Article  Google Scholar 

  29. European evidence-based guidelines on pancreatic cystic neoplasms. European Study Group on Cystic Tumours of the Pancreas. Gut. 2018;67:789–804.

    Google Scholar 

  30. Scheiman JM, Hwang JH, Moayyedi P. American gastroenterological association technical review on the diagnosis and management of asymptomatic neoplastic pancreatic cysts. Gastroenterology. 2015;148(4):824–48.e22.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Noriyoshi Fukushima .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2022 The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Fukushima, N. (2022). Mucinous Cystic Neoplasms. In: Makuuchi, M., et al. The IASGO Textbook of Multi-Disciplinary Management of Hepato-Pancreato-Biliary Diseases. Springer, Singapore. https://doi.org/10.1007/978-981-19-0063-1_11

Download citation

  • DOI: https://doi.org/10.1007/978-981-19-0063-1_11

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-19-0062-4

  • Online ISBN: 978-981-19-0063-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics