Microadenomatosis of the Endocrine Pancreas in Patients With and Without the Multiple Endocrine Neoplasia Type 1 Syndrome
Abstract
It has been suggested that microadenomatosis of the endocrine pancreas is a hallmark of the multiple endocrine neoplasia type 1 syndrome (MEN1). This study attempts to elucidate the relationship between pancreatic microadenomatosis and the MEN1 and von Hippel-Lindau (VHL) syndromes. Pancreatic tissue specimens from 37 patients (with either microadenomatosis or the MEN1 syndrome) were analyzed using immunohistochemistry, confocal laser scanning microscopy, and morphometric methods. The MEN1 and the VHL status were assessed on the basis of clinical criteria (all patients) and PCR-based mutational analysis (15 and 5 patients, respectively). Pancreatic microadenomatosis was found in 35 of 37 patients, 28 of whom fulfilled the clinicopathologic criteria and 13 the genetic criteria for MEN1, whereas none of the patients had evidence of a VHL syndrome. Microadenomas were present in 26 of the 28 MEN1 patients, and all these tumors were consistently multihormonal. Five of the 9 patients with microadenomatosis and no clinical evidence for MEN1 or VHL also lacked mutations for the respective genes. Five of these 9 patients suffered from hyperinsulinism and revealed multiple insulin-positive tumors. The other patients were nonsymptomatic and showed multiple glucagon-expressing neoplasms. In microadenomatosis patients with and without the MEN1 syndrome, a subset of morphologically normal-appearing islets showed increased endocrine cell proliferation. In conclusion, endocrine multihormonal…
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Minor criteria for adult-onset nesidioblastosis comprise islet hypertrophy, islet hyperplasia, lobulated islets, and macronucleoli within the β-cells.
“Apart from that, minor criteria comprise islet hypertrophy, islet hyperplasia, lobulated islets, and macronucleoli within the β -cells [22-24].”
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- Authors
- Anlauf, M., Schlenger, R., Perren, A., Bauersfeld, J., Koch, C. A., Dralle, H., Raffel, A., Knoefel, W. T., Weihe, E., Ruszniewski, P., Couvelard, A., Komminoth, P., Heitz, P. U., & Klöppel, G.
- Affiliations
- Christian-Albrechts-Universität zu Kiel (DE)
- University Hospital Zurich (CH)
- Leipzig University (DE)
- Heinrich Heine University Düsseldorf (DE)
- Philipps University of Marburg (DE)
- Corresponding author
- Anlauf, Martin
- Schlenger, Regina
- Bauersfeld, Juliane
- Ruszniewski, Philippe
- Klöppel, Günter
- ORCID
- Venue
- American Journal of Surgical Pathology
- Published
- 2006-05
- Volume
- 30
- Issue
- 5
- Pages
- 560-574
- ISSN
- 0147-5185 (ISSN-L)
- OpenAlex
- W1973333569
- Cited by
- 223
- References
- 0
- Citations by year
- 2026: 8 · 2025: 7 · 2024: 10 · 2023: 10 · 2022: 8 · 2021: 13 · 2020: 9 · 2019: 9 · 2018: 11 · 2017: 13 · 2016: 9 · 2015: 23 · 2014: 6 · 2013: 15 · 2012: 16
- FWCI
- 9.27
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The American Journal of Surgical Pathology (publishedVersion)
- PubMed (publishedVersion)
- Bern Open Repository and Information System (University of Bern) (submittedVersion)
- Topics
- Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Health Sciences › Medicine › Pathology and Forensic Medicine › Genetic factors in colorectal cancer
- Keywords
- multiple endocrine neoplasia type 1, von Hippel-Lindau disease, pancreatic neuroendocrine tumors, insulinoma, glucagonoma, hyperinsulinism, MEN1 gene mutation, VHL mutation
- MeSH terms
- Adult, Aged, DNA Mutational Analysis, Female, von Hippel-Lindau Disease, Humans, Immunohistochemistry, Adenoma, Islet Cell, Male, Middle Aged, Mutation, Pancreatic Neoplasms, Polymerase Chain Reaction, Microscopy, Confocal, Multiple Endocrine Neoplasia Type 1
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