{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T12:37:59Z","timestamp":1790685479053,"version":"4.1.0"},"reference-count":0,"publisher":"Bioscientifica","issue":"4","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2010,12]]},"abstract":"<jats:p>\n                    Pancreatic islet cell tumors (ICTs) occur as sporadic neoplasias or as a manifestation of multiple endocrine neoplasia type 1 (MEN1) and von Hippel\u2013Lindau disease (VHL). Molecular classification of ICTs is mandatory for timely diagnosis and surveillance. Systematic comparison of VHL-ICTs and sporadic ICTs has been lacking. Our registry-based approaches used the German NET-Registry with 259 patients with neuroendocrine tumors (NETs), who were primarily diagnosed with NETs, and the German VHL-Registry with 485 molecular genetically confirmed patients who had undergone magnetic resonance imaging or computed tomography of the abdomen. All patients provided blood DNA for testing of the\n                    <jats:italic>MEN1<\/jats:italic>\n                    and\n                    <jats:italic>VHL<\/jats:italic>\n                    genes for intragenic mutations and large deletions. In the NET-Registry, 9\/101 patients (8.9%) with ICTs had germline mutations, 8 in\n                    <jats:italic>MEN1<\/jats:italic>\n                    and 1 in\n                    <jats:italic>VHL<\/jats:italic>\n                    . In the VHL-Registry, prevalence of NETs was 52\/487 (10.6%), and all were ICTs. Interestingly, of those with\n                    <jats:italic>VHL<\/jats:italic>\n                    p.R167W, 47% developed ICTs, compared to 2% of those with p.Y98H. In total, there were 92 truly sporadic, i.e. mutation-negative ICT patients. Comparing these with the 53 VHL-ICT patients, the statistically significant differences were predominance of female gender (\n                    <jats:italic>P<\/jats:italic>\n                    =0.01), multifocal ICTs (\n                    <jats:italic>P<\/jats:italic>\n                    =0.0029), and lower malignancy rate (\n                    <jats:italic>P<\/jats:italic>\n                    &lt;0.001) in VHL-ICTs compared to sporadic cases. VHL was prevalent in &lt;0.5% of NETs, while NETs occur in \u223c10% of VHL, virtually exclusively as ICTs, which are rarely the first presentation. Patients with NETs should not be subjected to genetic testing of the\n                    <jats:italic>VHL<\/jats:italic>\n                    gene, unless they have multifocal ICTs, other VHL-associated tumors, and\/or a family history for VHL.\n                  <\/jats:p>","DOI":"10.1677\/erc-10-0037","type":"journal-article","created":{"date-parts":[[2010,7,26]],"date-time":"2010-07-26T22:46:56Z","timestamp":1280184416000},"page":"875-883","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":36,"title":["Systematic comparison of sporadic and syndromic pancreatic islet cell 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