Virchows Archiv

, Volume 458, Issue 1, pp 47–53 | Cite as

Glucagon expression in cystic pancreatic neuroendocrine neoplasms: an immunohistochemical analysis

  • Björn Konukiewitz
  • Tetsuji Enosawa
  • Günter Klöppel
Original Article


Pancreatic neuroendocrine neoplasms (P-NENs) are usually solid and only rarely cystic. Glucagon expression and the association with multiple endocrine neoplasia type 1 (MEN1) seem to be common in cystic P-NENs. In this study, we analyzed 404 P-NENs to gain information about the relative frequency of grossly cystic P-NENs and their association with glucagon production by the tumor cells. Three hundred forty-six solitary P-NENs and 58 P-NENs (>1 cm in diameter) from 35 patients with an MEN1 syndrome were studied. Immunostaining was performed for the four pancreatic hormones; 5.5% (19/346) of the sporadic P-NENs showed unilocular or multilocular cystic changes that were macroscopically detectable. Sixty-three percent of the solitary cystic P-NENs (versus 7% of the solitary non-cystic P-NENs) expressed predominantly glucagon. In MEN1-associated P-NENs, the relative frequency of cystic tumors was 10.3%, and all of them expressed glucagon. None of the glucagon-positive cystic P-NENs were associated with a glucagonoma syndrome. Solitary non-MEN1-associated and MEN1-associated cystic P-NENs are predominantly non-syndromic glucagon-expressing tumors. However, cystic insulinomas may also occur. Cyst formation seems to be related to hormone production.


Pancreatic neoplasms Neuroendocrine Cystic tumors Glucagon Insulin 



We are gratful to Maike Pacena for excellent technical assistance and Katherine Dege for editing the manuscript. We also thank all colleagues who provided consultation cases, which were included in the study. Special thanks are due to Prof. Wierich and Dr. Schmitz, Institute of Pathology, Recklinghausen, Germany, for providing macroscopic illustrations. This work was supported by Ipsen GmbH, Ettlingen, Germany in association with the German NET bank.

Conflict of interest

We declare that we have no conflict of interest.


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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Björn Konukiewitz
    • 1
  • Tetsuji Enosawa
    • 1
    • 2
  • Günter Klöppel
    • 1
    • 3
  1. 1.Department of PathologyUniversity of KielKielGermany
  2. 2.First Department of Pathology, Faculty of MedicineShowa UniversityTokyoJapan
  3. 3.Department of PathologyTechnical University of MünchenMunichGermany

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