{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T15:56:30Z","timestamp":1786982190224,"version":"build-2736575974"},"reference-count":22,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,5,25]],"date-time":"2021-05-25T00:00:00Z","timestamp":1621900800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,5,25]],"date-time":"2021-05-25T00:00:00Z","timestamp":1621900800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Endocrine"],"published-print":{"date-parts":[[2021,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>Data regarding the clinical management and follow-up of pancreatic neuroendocrine tumors (PanNETs) associated with Von Hippel\u2013Lindau (VHL) syndrome are limited. This study aimed to assess clinical presentation, genotype\u2013phenotype correlations, treatment and prognosis of PanNETs in a series of VHL syndrome patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Retrospective analysis of data of patients observed between 2005 and 2020.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Seventeen patients, including 12 probands and 5 relatives (mean age 30.8\u2009\u00b1\u200918.4; 7 males), were recruited. PanNETs were found in 13\/17 patients (77.5%) at a median age of 37 years: 4\/13 (30.7%) at the time of VHL diagnosis and 9 (69.3%) during follow up. Six (46.1%) PanNET patients underwent surgery, whereas seven were conservatively treated (mean tumor diameter: 40\u2009\u00b1\u200910.9 vs. 15\u2009\u00b1\u20095.3\u2009mm respectively). Four patients (30.7%) had lymph node metastases and a mean tumor diameter significantly larger than the nonmetastatic PanNETs (44.2\u2009\u00b1\u20099.3 vs. 17.4\u2009\u00b1\u20097\u2009mm,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.00049, respectively). Five (83.3%) operated patients had stable disease after a median follow up of 3 years whereas one patient showed liver metastases. Six (85.7%) non-resected PanNETs were stable after a median follow-up of 2 years, whereas one patient developed a new small PanNET and a slight increase in diameter of a pre-existing PanNET. No correlation was found between the type of germline mutation and malignant behavior of PanNETs.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>PanNETs are a common disease of the VHL syndrome and can be the presenting feature. Tumor size rather than genetic mutation is a prognostic factor of malignancy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s12020-021-02752-8","type":"journal-article","created":{"date-parts":[[2021,5,25]],"date-time":"2021-05-25T13:02:51Z","timestamp":1621947771000},"page":"180-187","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Clinical presentation, genotype\u2013phenotype correlations, and outcome of pancreatic neuroendocrine tumors in Von Hippel\u2013Lindau syndrome"],"prefix":"10.1007","volume":"74","author":[{"given":"F.","family":"Penitenti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Landoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Scardoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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The University and Hospital Trust Ethical Committee of Verona approved this study (n. 935 date 16\/09\/2020). This article does not contain any studies with animals performed by any of the authors.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}]}}