{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,8]],"date-time":"2025-09-08T06:40:18Z","timestamp":1757313618982},"reference-count":16,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2016,12,22]],"date-time":"2016-12-22T00:00:00Z","timestamp":1482364800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Head &amp; Neck"],"published-print":{"date-parts":[[2017,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>von Hippel\u2013Lindau (VHL)\u2010related tumors occurring outside the spectrum of VHL\u2010defining tumors are rare, and mucoepidermoid carcinoma (MEC) in the setting of VHL disease has not been described.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and Results<\/jats:title><jats:p>We describe a patient with confirmed VHL mutation who presented with a parotid mass and a history of 2 central nervous system (CNS) hemangioblastomas and 1 pheochromocytoma. Fine\u2010needle aspiration (FNA) of the mass suggested a benign Warthin tumor. The mass was resected and final pathology revealed a low\u2010grade MEC. Fluorescence in situ hybridization for the MECT1\/MAML2 fusion gene frequently associated with MEC was performed and was negative. Molecular testing of tumor cells displayed a likely \u201csecond hit\u201d <jats:italic>VHL<\/jats:italic> gene mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>There is a possible broader role of VHL mutations in tumorigenesis beyond the development of classically described VHL\u2010defining neoplasms. Our case also demonstrates the importance of always considering the possibility of a parotid malignancy in patients with VHL despite a benign FNA. \u00a9 2016 Wiley Periodicals, Inc. <jats:italic>Head Neck<\/jats:italic> 39: E51\u2013E54, 2017<\/jats:p><\/jats:sec>","DOI":"10.1002\/hed.24665","type":"journal-article","created":{"date-parts":[[2016,12,24]],"date-time":"2016-12-24T12:42:34Z","timestamp":1482583354000},"source":"Crossref","is-referenced-by-count":2,"title":["Case of parotid mucoepidermoid carcinoma: Expanding the spectrum of von Hippel\u2010Lindau\u2013related neoplasms"],"prefix":"10.1002","volume":"39","author":[{"given":"Michael H.","family":"Berger","sequence":"first","affiliation":[{"name":"Department of Otolaryngology \u2013 Head and Neck Surgery University of Miami Miller School of Medicine Miami Florida"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Darcy A.","family":"Kerr","sequence":"additional","affiliation":[{"name":"Department on Pathology University of Miami Miller School of Medicine Miami Florida"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Artur E.","family":"Rangel Filho","sequence":"additional","affiliation":[{"name":"Department on Pathology University of Miami Miller School of Medicine Miami Florida"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zoukaa B.","family":"Sargi","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology \u2013 Head and Neck Surgery University of Miami Miller School of Medicine Miami Florida"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,12,22]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01366952"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ejhg.2010.175"},{"key":"e_1_2_4_4_1","first-page":"298","article-title":"An association of parotid carcinoma with Von Hippel\u2013Lindau syndrome","volume":"51","author":"Nardi L","year":"1984","journal-title":"Mt Sinai J Med"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1209062"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.194.3.7862955"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.67.6.758"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/dc.23103"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bjorl.2015.03.007"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12105-013-0432-5"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoto.2015.3270"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3181de3021"},{"key":"e_1_2_4_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.20306"},{"key":"e_1_2_4_14_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A2520"},{"key":"e_1_2_4_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.24341"},{"key":"e_1_2_4_16_1","doi-asserted-by":"publisher","DOI":"10.5858\/2005-129-26-PISGFC"},{"key":"e_1_2_4_17_1","doi-asserted-by":"publisher","DOI":"10.1186\/1897-4287-3-4-171"}],"container-title":["Head &amp; Neck"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fhed.24665","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/hed.24665","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/hed.24665","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/hed.24665","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,6]],"date-time":"2023-09-06T09:12:44Z","timestamp":1693991564000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/hed.24665"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,12,22]]},"references-count":16,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2017,3]]}},"alternative-id":["10.1002\/hed.24665"],"URL":"https:\/\/doi.org\/10.1002\/hed.24665","archive":["Portico"],"relation":{},"ISSN":["1043-3074","1097-0347"],"issn-type":[{"value":"1043-3074","type":"print"},{"value":"1097-0347","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,12,22]]}}}