{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T21:07:11Z","timestamp":1774645631477,"version":"3.50.1"},"reference-count":0,"publisher":"Bioscientifica","issue":"11","license":[{"start":{"date-parts":[[2021,11,1]],"date-time":"2021-11-01T00:00:00Z","timestamp":1635724800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["ec.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,11,1]]},"abstract":"<jats:sec>\n<jats:title>Objective<\/jats:title>\n<jats:p>Pheochromocytomas are a hallmark feature of von Hippel\u2013Lindau disease (vHL). To our knowledge, this is the first systematic review with meta-analysis evaluating the frequency of pheochromocytomas and\/or paragangliomas (PPGLs) in patients with vHL, as well as among patients with different vHL subtypes.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Design<\/jats:title>\n<jats:p>Systematic review with meta-analysis.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>We searched on MEDLINE, Scopus, and Web of Science. We included primary studies assessing participants with vHL and reporting on the frequency of PPGL. We performed random-effects meta-analysis to quantitatively assess the frequency of PPGL, followed by meta-regression and subgroup analysis. Risk of bias analysis was performed to assess primary studies\u2019 methodological quality.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>We included 80 primary studies. In 4263 patients with vHL, the pooled frequency of PPGL was 19.4% (95% CI\u2009=\u200915.9\u201323.6%, <jats:italic>I<\/jats:italic><jats:sup>2<\/jats:sup> = 86.1%). The frequency increased to 60.0% in patients with vHL type 2 (95% CI\u2009=\u200953.4\u201366.3%, <jats:italic>I<\/jats:italic><jats:sup>2<\/jats:sup> = 54.6%) and was determined to be of 58.2% in patients with vHL type 2A (95% CI\u2009=\u200949.7\u201366.3%, <jats:italic>I<\/jats:italic><jats:sup>2<\/jats:sup> = 36.2%), compared to 49.8% in vHL type 2B (95% CI\u2009=\u200939.9\u201359.7%, <jats:italic>I<\/jats:italic><jats:sup>2<\/jats:sup> = 42.7%), and 84.1% in vHL type 2C (95% CI\u2009=\u200975.1\u201393.1%, <jats:italic>I<\/jats:italic><jats:sup>2<\/jats:sup> = 0%). In meta-regression analysis, more recent studies were associated with a higher frequency of PPGL. All studies had at least one internal validity item classified as 'high risk of bias,' with 13% studies having low risk of bias in all external validity items.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>PPGLs are a common manifestation of vHL. Despite methodological limitations and differences across primary studies, our results point to the importance of PPGL screening in patients with vHL.<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/ec-21-0294","type":"journal-article","created":{"date-parts":[[2021,10,1]],"date-time":"2021-10-01T14:42:16Z","timestamp":1633099336000},"page":"R293-R304","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-10","source":"Crossref","is-referenced-by-count":18,"title":["Pheochromocytomas and paragangliomas in von Hippel\u2013Lindau disease: not a needle in a haystack"],"prefix":"10.1530","volume":"10","author":[{"given":"Jo\u00e3o","family":"Castro-Teles","sequence":"first","affiliation":[{"name":"1Department of Biomedicine, Faculty of Medicine of the University of Porto, Porto, Portugal"}]},{"given":"Bernardo","family":"Sousa-Pinto","sequence":"additional","affiliation":[{"name":"2MEDCIDS, Department of Community Medicine, Information and Health Decision Sciences, Faculty of Medicine, University of Porto, Porto, Portugal"},{"name":"3CINTESIS, Center for Health Technology and Services Research, University of Porto, Porto, Portugal"}]},{"given":"Sandra","family":"Rebelo","sequence":"additional","affiliation":[{"name":"1Department of Biomedicine, Faculty of Medicine of the University of Porto, Porto, Portugal"},{"name":"4Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade (i3S), Universidade do Porto, Porto, Portugal"},{"name":"5Department of Clinical Pathology, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o (CHUSJ), Porto, Portugal"}]},{"given":"Duarte","family":"Pignatelli","sequence":"additional","affiliation":[{"name":"1Department of Biomedicine, Faculty of Medicine of the University of Porto, Porto, Portugal"},{"name":"4Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade (i3S), Universidade do Porto, Porto, Portugal"},{"name":"6Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Porto, Portugal"},{"name":"7Department of Endocrinology, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o (CHUSJ), Porto, Portugal"}]}],"member":"416","container-title":["Endocrine Connections"],"original-title":[],"link":[{"URL":"https:\/\/ec.bioscientifica.com\/view\/journals\/ec\/10\/11\/EC-21-0294.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/ec.bioscientifica.com\/downloadpdf\/journals\/ec\/10\/11\/EC-21-0294.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,16]],"date-time":"2021-11-16T12:06:33Z","timestamp":1637064393000},"score":1,"resource":{"primary":{"URL":"https:\/\/ec.bioscientifica.com\/view\/journals\/ec\/10\/11\/EC-21-0294.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,11,1]]},"references-count":0,"journal-issue":{"issue":"11"},"URL":"https:\/\/doi.org\/10.1530\/ec-21-0294","relation":{},"ISSN":["2049-3614"],"issn-type":[{"value":"2049-3614","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,11,1]]}}}