Glucagon expression in cystic pancreatic neuroendocrine neoplasms: an immunohistochemical analysis.

Abstract

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.

• Authors: Björn Konukiewitz, Tetsuji Enosawa, Günter Klöppel
• Journal: Virchows Archiv : an international journal of pathology (Virchows Arch) Vol. 458 Issue 1 Pg. 47-53 (Jan 2011) ISSN: 1432-2307 [Electronic] Germany
• PMID: 20922407 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Cadherins
• Pancreatic Hormones
• Platelet Endothelial Cell Adhesion Molecule-1
• beta Catenin
• Glucagon

Topics

• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Cadherins (metabolism)
• Child
• Child, Preschool
• Female
• Glucagon (metabolism)
• Humans
• Male
• Middle Aged
• Multiple Endocrine Neoplasia (epidemiology, metabolism, pathology)
• Neuroendocrine Tumors (epidemiology, metabolism, pathology)
• Pancreatic Hormones (metabolism)
• Pancreatic Neoplasms (epidemiology, metabolism, pathology)
• Platelet Endothelial Cell Adhesion Molecule-1 (metabolism)
• Prevalence
• Retrospective Studies
• Young Adult
• beta Catenin (metabolism)