Sectioning of the pancreas demonstrates multiple cysts without any clear communication with the pancreatic duct and no evidence of pancreatic duct dilatation

Diffuse Pancreatic Mucinous Cystic Neoplasm Treated by Total Pancreatectomy

Hongyi Chen, Julie Teague, Laurence Weinberg, Mehrdad Nikfarjam


Context Multifocal or diffuse mucinous cystic neoplasm are uncommon and may be difficult to distinguish from multifocal intra-ducal mucinous neoplasm or diffuse serous cystadenoma. Case report A forty-seven-year old lady with vague abdominal pain was noted to have cystic lesions ranging from 5 to 20 mm throughout her pancreas. The cysts had enlarged over several years of observation. There was no evidence of pancreatic duct dilatation or communication with the pancreatic duct on magnetic resonance imaging. Cyst fluid analysis for carcinoembryonic antigen and amylase were non-diagnostic. A total pancreatectomy was performed, with histology confirming numerous cysts lined by mucus producing cells, without obvious ovarian-like stroma. The stroma did however demonstrate positive staining for oestrogen receptor and smooth muscle actin. These findings were most consistent with a mucinous cystic neoplasm, despite the apparent absence of typical ovarian like stroma. Conclusion Multifocal or diffuse pancreatic mucinous cystic neoplasm are uncommon and may be suspected when imaging demonstrates multiple pancreatic cysts without communication with the pancreatic duct or pancreatic duct dilation. Surgical resection is indicated due to the increased risk of malignancy.

Image: Sectioning of the pancreas demonstrates multiple cysts without any clear communication with the pancreatic duct and no evidence of pancreatic duct dilatation.


Pancreatic Cyst; Pancreatectomy

Full Text:



Adsay NV, Klimstra DS, Compton CC. Cystic lesions of the pancreas. Introduction. Seminars in diagnostic pathology 2000; 17: 1-6.

Tanaka M, Fernandez-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: official journal of the International Association of Pancreatology (IAP) 2012; 12: 183-97. [PMID: 22687371]

Zamboni G, Hirabayashi K, Castelli P, Lennon AM. Precancerous lesions of the pancreas. Best practice & research Clinical gastroenterology 2013; 27: 299-322. [PMID: 23809247]

Reddy RP, Smyrk TC, Zapiach M, Levy MJ, Pearson RK, Clain JE, et al. Pancreatic mucinous cystic neoplasm defined by ovarian stroma: demographics, clinical features, and prevalence of cancer. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association 2004; 2: 1026-31. [PMID: 15551256]

Crippa S, Salvia R, Warshaw AL, Dominguez I, Bassi C, Falconi M, et al. Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients. Annals of surgery 2008; 247: 571-9. [PMID: 18362619]

Graziani R, Mautone S, Vigo M, Manfredi R, Opocher G, Falconi M. Spectrum of magnetic resonance imaging findings in pancreatic and other abdominal manifestations of Von Hippel-Lindau disease in a series of 23 patients: a pictorial review. JOP : Journal of the pancreas 2014; 15: 1-18. [PMID: 24413778]

Lee KS, Sekhar A, Rofsky NM, Pedrosa I. Prevalence of incidental pancreatic cysts in the adult population on MR imaging. The American journal of gastroenterology 2010; 105: 2079-84. [PMID: 20354507]

Nijs EL, Callahan MJ. Congenital and developmental pancreatic anomalies: ultrasound, computed tomography, and magnetic resonance imaging features. Seminars in ultrasound, CT, and MR 2007; 28: 395-401. [PMID: 17970555]

Goldsmith JD. Cystic neoplasms of the pancreas. American journal of clinical pathology 2003; 119: S3-16. [PMID: 12951841]

Antonini F, Fuccio L, Fabbri C, Macarri G, Palazzo L. Management of serous cystic neoplasms of the pancreas. Expert review of gastroenterology & hepatology 2014: 1-11. [PMID: 24981593]

Brugge WR, Lauwers GY, Sahani D, Fernandez-del Castillo C, Warshaw AL. Cystic neoplasms of the pancreas. The New England journal of medicine 2004; 351: 1218-26.

Batabyal P, Vander Hoorn S, Christophi C, Nikfarjam M. Association of diabetes mellitus and pancreatic adenocarcinoma: a meta-analysis of 88 studies. Annals of surgical oncology 2014; 21: 2453-62. [PMID: 24609291]

Warshaw AL, Compton CC, Lewandrowski K, Cardenosa G, Mueller PR. Cystic tumors of the pancreas. New clinical, radiologic, and pathologic observations in 67 patients. Annals of surgery. 1990; 212: 432-43. [PMID: 2171441]

Parra-Herran CE, Garcia MT, Herrera L, Bejarano PA. Cystic lesions of the pancreas: clinical and pathologic review of cases in a five year period. JOP : Journal of the pancreas 2010; 11: 358-64. [PMID: 20601810]

Asciutti S, Kanninen TT, Clerici G, Nardi E, Castellani D, GC DIR, et al. Acute pancreatitis with a mucinous cystoadenoma of the pancreas in pregnancy. Anticancer research 2010; 30: 1025-8.

Goh BK, Tan YM, Cheow PC, Chung YF, Chow PK, Wong WK, et al. Cystic neoplasms of the pancreas with mucin-production. European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology 2005; 31: 282-7.

Colovic R, Barisic G, Colovic N, Markovic V, Nadj G. Double mucinous cystadenoma of the pancreas associated with thecoma of the ovary. Acta chirurgica Iugoslavica 2002; 49: 95-7. [PMID: 12587492]

Wayne M, Gur D, Ascunce G, Abodessa B, Ghali V. Pancreatic mucinous cystic neoplasm with sarcomatous stroma metastasizing to liver: a case report and review of literature. World journal of surgical oncology 2013; 11: 100. [PMID: ]

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. The American journal of surgical pathology 1999; 23: 410-22. [PMID: 10199470]

Compagno J, Oertel JE. Mucinous cystic neoplasms of the pancreas with overt and latent malignancy (cystadenocarcinoma and cystadenoma). A clinicopathologic study of 41 cases. American journal of clinical pathology 1978; 69: 573-80. [PMID: 665578]



Article Metrics

Metrics Loading ...

Metrics powered by PLOS ALM


  • There are currently no refbacks.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

ISSN 1590-8577 | JOP is published by CAB - Center for Libraries at "Federico II" University of Naples using Open Journal System