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There was no family history of phaeochromocytomas or familial syndromes; however, a targeted genetic analysis for causes of familial phaeochromocytomas identified a heterozygous germline mutation in the <jats:italic>VHL<\/jats:italic> gene consistent with Von Hippel-Lindau syndrome. In this case, the identification of the VHL mutation led to careful screening and detection of clinically occult central nervous system hemangioblastomas and pancreatic neuroendocrine tumours. Verified genetic mutations facilitated best practices for long-term surveillance protocols, preconception counselling and screening of blood relatives. The patient responded well to surgical treatment and has ongoing multidisciplinary long-term surveillance.<\/jats:p>","DOI":"10.1136\/bcr-2018-225162","type":"journal-article","created":{"date-parts":[[2018,7,24]],"date-time":"2018-07-24T13:01:26Z","timestamp":1532437286000},"page":"bcr-2018-225162","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Unsuspected Von Hippel-Lindau syndrome in acute-onset resistant hypertension"],"prefix":"10.1136","volume":"2018","author":[{"given":"Gavasker A","family":"Sivaskandarajah","sequence":"first","affiliation":[{"name":"Department of Medicine, University of Saskatchewan College of Medicine, Saskatoon, Saskatchewan, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5793-7713","authenticated-orcid":false,"given":"Terra G","family":"Arnason","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Saskatchewan College of Medicine, Saskatoon, Saskatchewan, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2018,7,24]]},"reference":[{"key":"2025082723435180000_2018.jul24_1.bcr-2018-225162.1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1524-6175.2002.01452.x"},{"key":"2025082723435180000_2018.jul24_1.bcr-2018-225162.2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa020152"},{"key":"2025082723435180000_2018.jul24_1.bcr-2018-225162.3","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-09-1237"},{"key":"2025082723435180000_2018.jul24_1.bcr-2018-225162.4","doi-asserted-by":"publisher","DOI":"10.1373\/clinchem.2010.153320"},{"key":"2025082723435180000_2018.jul24_1.bcr-2018-225162.5","doi-asserted-by":"publisher","DOI":"10.1038\/jhh.2012.20"},{"key":"2025082723435180000_2018.jul24_1.bcr-2018-225162.6","doi-asserted-by":"crossref","first-page":"20140050","DOI":"10.1259\/bjr.20140050","article-title":"Pancreaticoduodenectomy: expected post-operative anatomy and complications","volume":"87","author":"McEvoy","year":"2014","journal-title":"Br J Radiol"},{"key":"2025082723435180000_2018.jul24_1.bcr-2018-225162.7","first-page":"B4763","article-title":"Von Hippel-Lindau disease (vHL). 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