{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T16:44:35Z","timestamp":1791218675829,"version":"4.1.0"},"reference-count":33,"publisher":"Wiley","issue":"S1","license":[{"start":{"date-parts":[[2015,7,14]],"date-time":"2015-07-14T00:00:00Z","timestamp":1436832000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100005972","name":"Deutsche Krebshilfe","doi-asserted-by":"crossref","award":["107995"],"award-info":[{"award-number":["107995"]}],"id":[{"id":"10.13039\/501100005972","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Direction G\u00e9n\u00e9rale de l'Organisation des Soins"},{"name":"Institut National du Cancer"},{"DOI":"10.13039\/501100004099","name":"Ligue Nationale contre le Cancer","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004099","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Head &amp; Neck"],"published-print":{"date-parts":[[2016,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Endolymphatic sac tumors (ELSTs) are, with a prevalence of up to 16%, a component of von Hippel\u2013Lindau (VHL) disease. Data from international registries regarding heritable fraction and characteristics, germline\n                      <jats:italic>VHL<\/jats:italic>\n                      mutation frequency, and prevalence are lacking.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Systematic registration of ELSTs from international centers of otorhinolaryngology and from multidisciplinary VHL centers\u2019 registries was performed. Molecular genetic analyses of the\n                      <jats:italic>VHL<\/jats:italic>\n                      gene were offered to all patients.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Our population\u2010based registry comprised 93 patients with ELST and 1789 patients with VHL. The prevalence of\n                      <jats:italic>VHL<\/jats:italic>\n                      germline mutations in apparently sporadic ELSTs was 39%. The prevalence of ELSTs in patients with VHL was 3.6%. ELST was the initial manifestation in 32% of patients with VHL\u2010ELST.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      Prevalence of ELST in VHL disease is much lower compared to the literature. VHL\u2010associated ELSTs can be the first presentation of the syndrome and mimic sporadic tumors, thus emphasizing the need of molecular testing in all presentations of ELST. \u00a9 2015 Wiley Periodicals, Inc.\n                      <jats:italic>Head Neck<\/jats:italic>\n                      <jats:bold>38:<\/jats:bold>\n                      673\u2013679, 2016\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hed.24067","type":"journal-article","created":{"date-parts":[[2015,4,13]],"date-time":"2015-04-13T10:31:12Z","timestamp":1428921072000},"source":"Crossref","is-referenced-by-count":54,"title":["Characterization of endolymphatic sac tumors and von Hippel\u2013Lindau disease in the International Endolymphatic Sac Tumor Registry"],"prefix":"10.1002","volume":"38","author":[{"given":"Birke","family":"Bausch","sequence":"first","affiliation":[{"name":"Second Department of Medicine Albert\u2013Ludwigs\u2010University of Freiburg Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Wellner","sequence":"additional","affiliation":[{"name":"Department of Surgery University Hospital Schleswig\u2013Holstein, Campus Luebeck Luebeck Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathieu","family":"Peyre","sequence":"additional","affiliation":[{"name":"Center Expert National Cancers Rares PREDIR, AP\u2010HP INCa, H\u00f4pital de Bic\u00eatre Le Kremlin\u2010Bic\u00eatre France"},{"name":"G\u00e9n\u00e9tique Oncologique EPHE, INSERM U 753, Facult\u00e9 de M\u00e9decine Paris Sud and Institut de Canc\u00e9rologie Gustave Roussy, Villejuif, France and Service de Neurochirurgie, AP\u2010HP H\u00f4pital Beaujon Clichy France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten C.","family":"Boedeker","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology University Medical Center, Albert\u2013Ludwigs\u2010University Freiburg"},{"name":"HELIOS Hanseklinikum Stralsund Stralsund Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederik J.","family":"Hes","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics Leiden University Medical Center Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariagiulia","family":"Anglani","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology University of Padova Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose M.","family":"de Campos","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery IIS \u2013 Fundaci\u00f3n Jim\u00e9nez D\u00edaz. UAM Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Kanno","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Yokohama City University Yokohama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eamonn R.","family":"Maher","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics University of Cambridge and NIHR Cambridge Biomedical Research Center Cambridge United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Krauss","sequence":"additional","affiliation":[{"name":"Department of Radiology Albert\u2013Ludwigs\u2010University of Freiburg Freiburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriela","family":"Sans\u00f3","sequence":"additional","affiliation":[{"name":"Centro de Investigaciones Endocrinol\u00f3gicas (CONICET), Hospital de Ni\u00f1os \u201cR. 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