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Multiple endocrine neoplasia type 1 (MEN1) : loss of one MEN1 allele in tumors and monohormonal endocrine cell clusters but not in islet hyperplasia of the pancreas.

Perren, Aurel (författare)
Department of Pathology, Institute of Surgical Pathology, University Hospital, CH-8091 Zürich, Switzerland / Institute of Pathology, Technische Universitaet Muenchen, D-81675 Munich, Germany
Anlauf, Martin (författare)
Department of Pathology, University of Kiel, D-24105 Kiel, Germany
Henopp, Tobias (författare)
Department of Pathology, University of Kiel, D-24105 Kiel, Germany
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Rudolph, Thomas (författare)
Department of Pathology, Institute of Surgical Pathology, University Hospital, CH-8091 Zürich, Switzerland
Schmitt, Anja (författare)
Department of Pathology, Institute of Surgical Pathology, University Hospital, CH-8091 Zürich, Switzerland
Raffel, Andreas (författare)
Department of General, Visceral, and Pediatric Surgery, University of Düsseldorf, D-40225 Düsseldorf, Germany
Gimm, Oliver (författare)
Department of General, Visceral, and Vascular Surgery, University of Halle, D-06120 Halle, Germany
Weihe, Eberhard (författare)
Departments of Molecular Neuroscience and Anatomy and Cell Biology, University of Marburg, D-35037 Marburg, Germany
Knoefel, Wolfram T (författare)
Department of General, Visceral, and Pediatric Surgery, University of Düsseldorf, D-40225 Düsseldorf, Germany
Dralle, Henning (författare)
Department of General, Visceral, and Vascular Surger, University of Halle, D-06120 Halle, Germany
Heitz, Philipp U (författare)
Department of Pathology, Institute of Surgical Pathology, University Hospital, CH-8091 Zürich, Switzerland
Komminoth, Paul (författare)
Institute of Pathology, Stadtspital Triemli, CH-8063 Zurich, Switzerland
Klöppel, Günter (författare)
Department of Pathology, University of Kiel, D-24105 Kiel, Germany
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 (creator_code:org_t)
The Endocrine Society, 2007
2007
Engelska.
Ingår i: Journal of Clinical Endocrinology and Metabolism. - : The Endocrine Society. - 0021-972X .- 1945-7197. ; 92:3, s. 1118-28
  • Tidskriftsartikel (refereegranskat)
Abstract Ämnesord
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  • CONTEXT: The occurrence of multiple small pancreatic endocrine tumors in patients suffering from multiple endocrine neoplasia type 1 (MEN1) represents a unique possibility to study early neoplasms and their potential precursor lesions. To date, it is unknown whether small islet-like endocrine cell clusters found in MEN1 patients are neoplastic or rather hyperplastic. It is also unclear whether microadenomas develop from islets.DESIGN: We hypothesized that monohormonal endocrine cell clusters observed in MEN1 patients are small neoplasms with loss of heterozygosity of the MEN1 locus. Using a technique combining fluorescence in situ hybridization of the MEN1 locus and the centromeric region of chromosome 11q with hormone immunostaining, we examined resection specimens from four MEN1 patients. We focused our investigations on the following: 1) typical microadenomas; 2) monohormonal endocrine cell clusters; 3) endocrine and exocrine structures entrapped in microadenomas; and 4) morphologically normal islets.RESULTS: Loss of one MEN1 allele was found in all 27 microadenomas and 19 of 20 (95%) monohormonal endocrine cell clusters. By contrast, it was absent in islets and ductal or acinar structures. Our results indicate that monohormonal endocrine cell clusters represent a minute form of microadenomas.CONCLUSION: The frequent presence of single nonneoplastic insulin cells in microadenomas and the occurrence of microadenomas in islets suggest an islet origin of microadenomas. Islet hyperplasia does not seem to be an obligatory stage in pancreatic MEN1-associated tumor development.

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