{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T10:12:59Z","timestamp":1791972779255,"version":"4.3.4"},"reference-count":64,"publisher":"Bioscientifica","issue":"9","content-domain":{"domain":["erc.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2018,9]]},"abstract":"<jats:p>\n                    Pancreatic neuroendocrine tumors (PanNETs) are rare in von Hippel\u2013Lindau disease (VHL) but cause serious morbidity and mortality. Management guidelines for VHL-PanNETs continue to be based on limited evidence, and survival data to guide surgical management are lacking. We established the European-American-Asian-VHL-PanNET-Registry to assess data for risks for metastases, survival and long-term outcomes to provide best management recommendations. Of 2330 VHL patients, 273 had a total of 484 PanNETs. Median age at diagnosis of PanNET was 35 years (range 10\u201375). Fifty-five (20%) patients had metastatic PanNETs. Metastatic PanNETs were significantly larger (median size 5 vs 2\u2009cm;\n                    <jats:italic>P<\/jats:italic>\n                    \u2009&lt;\u20090.001) and tumor volume doubling time (TVDT) was faster (22 vs 126 months;\n                    <jats:italic>P<\/jats:italic>\n                    \u2009=\u20090.001). All metastatic tumors were \u22652.8\u2009cm. Codons 161 and 167 were hotspots for\n                    <jats:italic>VHL<\/jats:italic>\n                    germline mutations with enhanced risk for metastatic PanNETs. Multivariate prediction modeling disclosed maximum tumor diameter and TVDT as significant predictors for metastatic disease (positive and negative predictive values of 51% and 100% for diameter cut-off \u22652.8\u2009cm, 44% and 91% for TVDT cut-off of \u226424 months). In 117 of 273 patients, PanNETs &gt;1.5\u2009cm in diameter were operated. Ten-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs &lt;2.8\u2009cm vs \u22652.8\u2009cm (94% vs 85% by 10 years;\n                    <jats:italic>P<\/jats:italic>\n                    \u2009=\u20090.020; 80% vs 50% at 10 years;\n                    <jats:italic>P<\/jats:italic>\n                    \u2009=\u20090.030). This study demonstrates that patients with PanNET approaching the cut-off diameter of 2.8\u2009cm should be operated. Mutations in exon 3, especially of codons 161\/167 are at enhanced risk for metastatic PanNETs. Survival is significantly longer in operated non-metastatic VHL-PanNETs.\n                  <\/jats:p>","DOI":"10.1530\/erc-18-0100","type":"journal-article","created":{"date-parts":[[2018,5,10]],"date-time":"2018-05-10T12:15:16Z","timestamp":1525954516000},"page":"783-793","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-1","source":"Crossref","is-referenced-by-count":59,"title":["Preventive medicine of von Hippel\u2013Lindau disease-associated pancreatic neuroendocrine tumors"],"prefix":"10.1530","volume":"25","author":[{"given":"Tobias","family":"Krauss","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alfonso Massimiliano","family":"Ferrara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thera 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