[Skip to Navigation]
Sign In
Special Feature
January 19, 2009

Image of the Month—Diagnosis

Arch Surg. 2009;144(1):89-90. doi:10.1001/archsurg.2008.527-b

Answer: Low-Grade, Nonfunctioning Cystic Pancreatic Endocrine Neoplasm

Cystic neoplasms of the pancreas account for about 1% of all pancreatic tumors. Cystic neoplasms of the pancreas may be classified into 3 categories. True neoplastic cysts, the cystadenomas and cystadenocarcinomas arising from the ductal epithelium, account for 90% of cystic pancreatic tumors.1Intraductal tumors like intraductal pancreatic mucinous neoplasm and intraductal pancreatic endocrine neoplasm (PEN) may demonstrate cystic dilatation of the distal pancreatic ducts. Solid tumors of the pancreas like ductal adenocarcinoma, acinar cell carcinoma, and PEN may very rarely undergo cystic degeneration following central necrosis.2Truly cystic PEN, however, has also been reported.3

Pancreatic endocrine neoplasms account for less than 5% of all cystic pancreatic neoplasms.3The majority of cystic PENs are nonfunctional.4They generally reach a larger size than their solid counterparts.5They most commonly occur in the body and tail of the pancreas in patients between the ages of 50 and 60 years.3They are equally distributed between men and women.

One of the most controversial aspects of PENs is the prediction of biologic behavior. Factors that adversely correlate with clinical outcome include tumor size (>3 cm), capsular and vascular invasion, and a mitotic rate greater than 2 of 10 per high-power field. Both in cytology and on tissue sections, immunohistochemical analysis is very helpful in distinguishing endocrine, ductal, and acinar tumors. Most, if not all, PENs show positivity for general endocrine markers, including synaptophysin and chromogranin. The tumor in our patient exhibited all the clinicopathological features of a low-grade, nonfunctioning cystic pancreatic endocrine neoplasm.

About a third of PENs are malignant. Cystic PENs are composed of cystic spaces that may be lined by characteristic islet cells or may contain necrotic material. All PENs should be resected, both to provide relief of symptoms and to detect the presence or absence of malignancy.3

Return to 0

Return to Quiz Case.

Box Section Ref ID


The Editor welcomes contributions to the Image of the Month. Manuscripts should be submitted via our online manuscript submission and review system (http://manuscripts.archsurg.com). Articles and photographs accepted will bear the contributor's name. Manuscript criteria and information are per the Instructions for Authors for Archives of Surgery (http://archsurg.ama-assn.org/misc/ifora.dtl). No abstract is needed, and the manuscript should be no more than 3 typewritten pages. There should be a brief introduction, 1 multiple-choice question with 4 possible answers, and the main text. No more than 2 photographs should be submitted. There is no charge for reproduction and printing of color illustrations.

Correspondence:Adhish Basu, MS, MRCS, Department of General Surgery, Providence Hospital and Medical Centers, 16001 W 9 Mile Rd, Southfield, MI 48075 (adhishbasu@rediffmail.com).

Accepted for Publication:March 11, 2007.

Author Contributions:Study concept and design: Basu and Sistla. Acquisition of data: Basu and Iyengar. Analysis and interpretation of data: Basu. Drafting of the manuscript: Basu. Critical revision of the manuscript for important intellectual content: Sistla and Iyengar. Administrative, technical, and material support: Basu and Iyengar. Study supervision: Sistla and Iyengar.

Financial Disclosure:None reported.

Le Borgne  J Cystic tumors of the pancreas.  Br J Surg 1998;85 (5) 577- 579PubMedGoogle ScholarCrossref
Adsay  NVKlimstra  DS Cystic forms of typically solid pancreatic tumors.  Semin Diagn Pathol 2000;17 (1) 81- 88PubMedGoogle Scholar
Box  JCDouglass  HO Management of cystic neoplasms of the pancreas.  Am Surg 2000;66 (5) 495- 501PubMedGoogle Scholar
Schwartz  RWMunfakh  NAZweng  TNStrodel  WELee  EThompson  NW Nonfunctioning cystic neuroendocrine neoplasms of the pancreas.  Surgery 1994;115 (5) 645- 649PubMedGoogle Scholar
Goh  BKOoi  LLTan  YM  et al.  Clinico-pathological features of cystic pancreatic endocrine neoplasms and a comparison with their solid counterparts.  Eur J Surg Oncol 2006;32 (5) 553- 556PubMedGoogle ScholarCrossref