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Open Access Green as soon as Postprint is submitted to ZB.
Glucagon cell adenomatosis: A newly recognized disease of the endocrine pancreas.
J. Clin. Endocrinol. Metab. 94, 213-217 (2009)
Glucagon-producing tumors are either solitary neoplasms of the pancreas, occasionally associated with a glucagonoma syndrome, or multiple neoplasms associated with multiple endocrine neoplasia type 1 (MEN1). We observed a previously undescribed multicentric glucagon-producing tumor disease that is not related to MEN1. Methods: Pancreatic tissue from four patients showing multiple neuroendocrine microadenomas and in two cases also macrotumors was screened for hormones using immunohistochemical and morphometric methods. MEN1, VHL and p27 germline and somatic mutation analysis was performed. Deletion of MEN1 (11q13), VHL (3p25) and the centromere 11 and 3 gene locus was determined by fluorescence in situ hybridization (FISH). DNA copy number changes were studied using array comparative genomic hybridization (CGH). Results: The pancreatic tissue from the four patients contained more than 870 microadenomas and 10 macrotumors, all of which expressed exclusively glucagon and none of which showed evidence of malignancy. In addition, many islets were unusually large and showed glucagon cell hyperplasia. There was no clinical or molecular evidence of any hereditary tumor disease, and changes in the MEN1 gene were only seen in individual tumors. Array CGH of one macrotumor and 20 pooled microadenomas revealed a homogeneous diploid chromosome set. Conclusions: The findings are sufficiently distinctive to suggest a new neoplastic disease of the endocrine pancreas that we recommend calling glucagon cell adenomatosis. Clinically, this disease may be an incidental finding, or it may lead to a glucagonoma syndrome.
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Publication type
Article: Journal article
Document type
Scientific Article
Keywords
pancreatic endocrine tumor; p27; microadenomatosis; MEN1; glucagon cells; endocrine precursor lesion; VHL
ISSN (print) / ISBN
0021-972X
e-ISSN
1945-7197
Quellenangaben
Volume: 94,
Issue: 1,
Pages: 213-217
Publisher
Endocrine Society
Publishing Place
Bethesda, Md.
Non-patent literature
Publications
Reviewing status
Peer reviewed
Institute(s)
Institute of Pathology (PATH)