{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T22:10:29Z","timestamp":1793830229903,"version":"4.6.1"},"reference-count":25,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,11,26]],"date-time":"2019-11-26T00:00:00Z","timestamp":1574726400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2019,11,26]],"date-time":"2019-11-26T00:00:00Z","timestamp":1574726400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2019,11,26]],"date-time":"2019-11-26T00:00:00Z","timestamp":1574726400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["World j. surg."],"published-print":{"date-parts":[[2020,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Approximately 30% of patients with apparent sporadic pheochromocytoma (Pheo) may later prove to have an inherited predisposition syndrome, most commonly Von Hippel\u2013Lindau (VHL) disease. Our aim was to compare the clinical and biochemical features of Pheo in VHL to those in sporadic disease to identify differences that may be used to guide management and surveillance of Pheo in VHL patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data of all patients who had adrenalectomy for histologic Pheo from 2000 to 2018 (QIIRB1749) were retrospectively reviewed. VHL patients were diagnosed by standard clinical criteria and\/or genetic testing. Patients were classified as having sporadic Pheo (sPheo) if they had no family\/personal history consistent with an associated genetic disorder and\/or had negative testing for <jats:italic>VHL, MAX, MEN1, NF1, RET<\/jats:italic> and <jats:italic>SDHAF2\/B\/C\/D\/A<\/jats:italic> mutations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 175 patients, 38 (22%) had VHL and 137 (78%) had sPheo including 27 (20%) with negative genetic testing. Compared to sPheo, VHL Pheo patients were younger (mean 25.9 vs. 51.2 years, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), less symptomatic (55% vs. 72%, <jats:italic>p<\/jats:italic>\u2009=\u20090.074), less hypertensive (46% vs. 64%, <jats:italic>p<\/jats:italic>\u2009=\u20090.043) and were more likely to have normal plasma metanephrines (85% vs. 25%, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). VHL\u2010related Pheos were smaller (median 2.8\u00a0cm vs. 4.4\u00a0cm, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001) but more often multifocal (&gt;1 adrenal Pheo) (16% vs. 0%, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). Recurrence &gt;6\u00a0months from initial resection was common in VHL (40% vs. 0%, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), median time to recurrence 15 years, (range 1\u201338 years).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Compared to those with sporadic Pheo, patients with VHL are more likely to be young, asymptomatic and normotensive and to have small, multifocal, normetanephrine\u2010secreting tumors. Because recurrence is common in VHL and arises up to 38 years later, continued surveillance is advised.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s00268-019-05299-y","type":"journal-article","created":{"date-parts":[[2019,11,26]],"date-time":"2019-11-26T19:02:48Z","timestamp":1574794968000},"page":"570-577","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Clinical and Biochemical Features of Pheochromocytoma Characteristic of Von Hippel\u2013Lindau Syndrome"],"prefix":"10.1002","volume":"44","author":[{"given":"Shimena R.","family":"Li","sequence":"first","affiliation":[{"name":"Department of General Surgery University of Pittsburgh Medical Center Kaufman Building, Suite 101, 3471 Fifth Ave 15213 Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina J.","family":"Nicholson","sequence":"additional","affiliation":[{"name":"Department of General Surgery University of Pittsburgh Medical Center Kaufman Building, Suite 101, 3471 Fifth Ave 15213 Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly L.","family":"Mccoy","sequence":"additional","affiliation":[{"name":"Division of Endocrine Surgery University of Pittsburgh Medical Center Kaufman Building, Suite 101, 3471 Fifth Ave 15213 Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sally E.","family":"Carty","sequence":"additional","affiliation":[{"name":"Division of Endocrine Surgery University of Pittsburgh Medical Center Kaufman Building, Suite 101, 3471 Fifth Ave 15213 Pittsburgh PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linwah","family":"Yip","sequence":"additional","affiliation":[{"name":"Division of Endocrine Surgery University of Pittsburgh Medical Center Kaufman Building, Suite 101, 3471 Fifth Ave 15213 Pittsburgh PA 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