{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T10:47:14Z","timestamp":1780051634595,"version":"3.53.1"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,12,2]],"date-time":"2021-12-02T00:00:00Z","timestamp":1638403200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2021,12,2]],"date-time":"2021-12-02T00:00:00Z","timestamp":1638403200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2021,12,2]],"date-time":"2021-12-02T00:00:00Z","timestamp":1638403200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["World j. surg."],"published-print":{"date-parts":[[2022,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To compare clinical, biochemical, tumoural and mutational characteristics of Von Hippel Lindau Syndrome (VHL)\u2010associated pheochromocytoma (PCC) to multiple endocrine neoplasia 2A (MEN2A)\u2010associated pheochromocytoma.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design<\/jats:title><jats:p>Retrospective study design in a tertiary health care centre in Northern India.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 47 patients with biochemical and histologically proven pheochromocytoma\/paraganglioma (PCC\/PGL): 29 associated with VHL and 18 with MEN2A, were divided in two cohorts, respectively. Analysis of their medical records along with a prospective follow\u2010up was done.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There were more children\u2009&lt;19 years in VHL group (13 vs 1). Despite majority of VHL\u2010PCC showing elevation of normetanephrine (NMN) (93%) as compared to MEN2A\u2010PCC (22.2%), 75.8% presented with hypertension as compared to MEN2A (33.3%). The average size of VHL\u2010PCC tumours was 5.66\u00a0cm. VHL\u2010PCC as compared to MEN2A\u2010PCC were multifocal (75% vs 61.1%), bilateral synchronous (72.4 vs 61.1%) and extra\u2010adrenal (17.2% vs 0%). Both VHL (24%) and MEN2A\u2010PCC (27.7%) showed multiple nodules, but more MEN2A PCC showed extra\u2010tumoural hyperplasia (44.4% vs. 6.8%). In VHL, the commonest mutation (<jats:italic>n<\/jats:italic>\u2009=\u200917) was missense mutation with a hot spot on exon 3, while in MEN2A\u2010PCC majority (66.6%) had 634\u00a0mutation in exon 11 and only 2 patients had the rare 611\u00a0mutation in exon 10.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In contrast to world literature, our study suggests Indian VHL\u2010PCC can be symptomatic in spite of noradrenergic phenotype, large in size and multifocal. Multiple nodules in VHL\u2010PCC could increase risk of recurrence following subtotal adrenalectomy.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s00268-021-06393-w","type":"journal-article","created":{"date-parts":[[2021,12,2]],"date-time":"2021-12-02T17:03:29Z","timestamp":1638464609000},"page":"591-599","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Clinical, Biochemical, Tumoural and Mutation Profile of VHL\u2010 and MEN2A\u2010Associated Pheochromocytoma: A Comparative Study"],"prefix":"10.1002","volume":"46","author":[{"given":"Mallika","family":"Dhanda","sequence":"first","affiliation":[{"name":"Department of Endocrine Surgery Sanjay Gandhi Postgraduate Institute of Medical Sciences Raibareli Rd 226014 Lucknow Uttar Pradesh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amit","family":"Agarwal","sequence":"additional","affiliation":[{"name":"Department of Endocrine Surgery Sanjay Gandhi Postgraduate Institute of Medical Sciences Raibareli Rd 226014 Lucknow Uttar Pradesh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kausik","family":"Mandal","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics Sanjay Gandhi Postgraduate Institute of Medical Sciences Raibareli Rd 226014 Lucknow Uttar Pradesh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sushil","family":"Gupta","sequence":"additional","affiliation":[{"name":"Department of Endocrinology Sanjay Gandhi Postgraduate Institute of Medical Sciences Raibareli Rd 226014 Lucknow Uttar Pradesh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Sabaretnam","sequence":"additional","affiliation":[{"name":"Department of Endocrine Surgery Sanjay Gandhi Postgraduate Institute of Medical Sciences Raibareli Rd 226014 Lucknow Uttar Pradesh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gyan","family":"Chand","sequence":"additional","affiliation":[{"name":"Department of Endocrine Surgery Sanjay Gandhi Postgraduate Institute of Medical Sciences Raibareli Rd 226014 Lucknow Uttar Pradesh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anjali","family":"Mishra","sequence":"additional","affiliation":[{"name":"Department of Endocrine Surgery Sanjay Gandhi Postgraduate Institute of Medical Sciences Raibareli Rd 226014 Lucknow Uttar Pradesh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaurav","family":"Agarwal","sequence":"additional","affiliation":[{"name":"Department of Endocrine Surgery Sanjay Gandhi Postgraduate Institute of Medical Sciences Raibareli Rd 226014 Lucknow Uttar Pradesh India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saroj Kanta","family":"Mishra","sequence":"additional","affiliation":[{"name":"Department of Endocrine 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