PMID- 20922407 OWN - NLM STAT- MEDLINE DCOM- 20110210 LR - 20211020 IS - 1432-2307 (Electronic) IS - 0945-6317 (Linking) VI - 458 IP - 1 DP - 2011 Jan TI - Glucagon expression in cystic pancreatic neuroendocrine neoplasms: an immunohistochemical analysis. PG - 47-53 LID - 10.1007/s00428-010-0985-6 [doi] AB - 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. FAU - Konukiewitz, Bjorn AU - Konukiewitz B AD - Department of Pathology, University of Kiel, Kiel, Germany. FAU - Enosawa, Tetsuji AU - Enosawa T FAU - Kloppel, Gunter AU - Kloppel G LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20101005 PL - Germany TA - Virchows Arch JT - Virchows Archiv : an international journal of pathology JID - 9423843 RN - 0 (Cadherins) RN - 0 (Pancreatic Hormones) RN - 0 (Platelet Endothelial Cell Adhesion Molecule-1) RN - 0 (beta Catenin) RN - 9007-92-5 (Glucagon) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Cadherins/metabolism MH - Child MH - Child, Preschool MH - Female MH - Glucagon/*metabolism MH - Humans MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia/epidemiology/*metabolism/pathology MH - Neuroendocrine Tumors/epidemiology/*metabolism/pathology MH - Pancreatic Hormones/metabolism MH - Pancreatic Neoplasms/epidemiology/*metabolism/pathology MH - Platelet Endothelial Cell Adhesion Molecule-1/metabolism MH - Prevalence MH - Retrospective Studies MH - Young Adult MH - beta Catenin/metabolism EDAT- 2010/10/06 06:00 MHDA- 2011/02/11 06:00 CRDT- 2010/10/06 06:00 PHST- 2010/08/30 00:00 [received] PHST- 2010/09/16 00:00 [accepted] PHST- 2010/09/15 00:00 [revised] PHST- 2010/10/06 06:00 [entrez] PHST- 2010/10/06 06:00 [pubmed] PHST- 2011/02/11 06:00 [medline] AID - 10.1007/s00428-010-0985-6 [doi] PST - ppublish SO - Virchows Arch. 2011 Jan;458(1):47-53. doi: 10.1007/s00428-010-0985-6. Epub 2010 Oct 5.