{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,9]],"date-time":"2025-09-09T21:51:23Z","timestamp":1757454683283,"version":"3.37.3"},"reference-count":45,"publisher":"The Endocrine Society","issue":"9","license":[{"start":{"date-parts":[[2023,3,10]],"date-time":"2023-03-10T00:00:00Z","timestamp":1678406400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100006285","name":"Magnus Bergvall Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006285","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,8,18]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Context<\/jats:title>\n                  <jats:p>Adrenal medullary hyperplasia (AMH) is a rare, incompletely described disorder of the adrenal medulla that is associated with catecholamine excess.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>To increase knowledge about AMH by reviewing the reported cases of this disorder.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Design<\/jats:title>\n                  <jats:p>Systematic review and meta-analysis of the genotype\/phenotype relationship in all reported cases of AMH.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Setting<\/jats:title>\n                  <jats:p>Literature review and analysis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Patients or Other Participants<\/jats:title>\n                  <jats:p>All cases of AMH published to date.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Main Outcome Measure(s)<\/jats:title>\n                  <jats:p>Characteristics of AMH cases and genotype-phenotype relationships.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>A total of 66 patients, median age of 48 years, were identified from 29 reports. More than one-half were male (n = 39, 59%). The majority had unilateral (73%, n = 48) disease; 71% (n = 47) were sporadic and 23% (n = 15) were associated with the MEN2. Most (91%, n = 60) displayed signs and symptoms of excess catecholamine secretion, particularly hypertension. Elevated catecholamine concentrations (86%, n = 57) and adrenal abnormalities on imaging were common (80%, n = 53). More than one-half (58%, n = 38) had concurrent tumors: pheochromocytoma (42%, n = 16\/38); medullary thyroid cancer (24%, n = 9\/38); and adrenocortical adenoma (29%, n = 11\/38). Most (88%, n = 58) underwent adrenalectomy with 45\/58 achieving symptom resolution. Adrenalectomy was less common in patients under 40 years and those with bilateral disease (both P &amp;lt; .05).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>AMH may be sporadic or associated with MEN2, most have catecholamine excess and imaging abnormalities. Unilateral involvement is more common. Most reported patients have been treated with adrenalectomy, which is usually curative with regard to catecholamine hypersecretion.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/clinem\/dgad121","type":"journal-article","created":{"date-parts":[[2023,3,10]],"date-time":"2023-03-10T08:26:42Z","timestamp":1678436802000},"page":"e885-e892","source":"Crossref","is-referenced-by-count":3,"title":["Adrenal Medullary Hyperplasia: A Systematic Review and Meta-analysis"],"prefix":"10.1210","volume":"108","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9824-2144","authenticated-orcid":false,"given":"Rafal","family":"Ganni","sequence":"first","affiliation":[{"name":"School of Medicine, Sydney, The University of Notre Dame , Darlinghurst, NSW 2010 , Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David J","family":"Torpy","sequence":"additional","affiliation":[{"name":"Endocrine and Metabolic Unit, Royal Adelaide Hospital , Adelaide, SA , Australia"},{"name":"Faculty of Medicine, University of Adelaide , Adelaide, SA , Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5622-6987","authenticated-orcid":false,"given":"Henrik","family":"Falhammar","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Karolinska University Hospital , SE-17176 Stockholm , Sweden"},{"name":"Department of Molecular Medicine and Surgery, Karolinska Institutet , SE-17176 Stockholm , Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6270-747X","authenticated-orcid":false,"given":"R","family":"Louise Rushworth","sequence":"additional","affiliation":[{"name":"School of Medicine, Sydney, The University of Notre Dame , Darlinghurst, NSW 2010 , Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"80","published-online":{"date-parts":[[2023,3,10]]},"reference":[{"issue":"9","key":"2023081819563595500_dgad121-B1","doi-asserted-by":"crossref","first-page":"e236209","DOI":"10.1136\/bcr-2020-236209","article-title":"Adrenal medullary hyperplasia mimicking pheochromocytoma","volume":"13","author":"Montebello","year":"2020","journal-title":"BMJ Case Reports CP"},{"issue":"1","key":"2023081819563595500_dgad121-B2","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/j.amjms.2020.06.031","article-title":"Adrenal medullary hyperplasia: an under the radar cause of endocrine hypertension","volume":"363","author":"Mejia","year":"2022","journal-title":"Am J Med Sci"},{"issue":"4","key":"2023081819563595500_dgad121-B3","doi-asserted-by":"crossref","first-page":"587","DOI":"10.4103\/IJPM.IJPM_707_17","article-title":"Adrenal medullary hyperplasia with coexistent cerebral angiomas","volume":"61","author":"Gupta","year":"2018","journal-title":"Ind J Pathol Microbiol"},{"issue":"8","key":"2023081819563595500_dgad121-B4","doi-asserted-by":"crossref","first-page":"1518","DOI":"10.1210\/js.2019-00200","article-title":"Presentation, treatment, histology, and outcomes in adrenal medullary hyperplasia compared with pheochromocytoma","volume":"3","author":"Falhammar","year":"2019","journal-title":"J Endocr Soc"},{"key":"2023081819563595500_dgad121-B5","doi-asserted-by":"publisher","first-page":"752410","DOI":"10.1155\/2014\/752410","article-title":"Diagnosis and treatment of adrenal medullary hyperplasia: experience from 12 cases","volume":"2014","author":"Yang","year":"2014","journal-title":"Int J Endocrinol"},{"issue":"4","key":"2023081819563595500_dgad121-B6","doi-asserted-by":"crossref","first-page":"264","DOI":"10.7326\/0003-4819-151-4-200908180-00135","article-title":"Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement","volume":"151","author":"Moher","year":"2009","journal-title":"Ann Intern Med"},{"issue":"3","key":"2023081819563595500_dgad121-B7","doi-asserted-by":"crossref","first-page":"433","DOI":"10.4293\/108680813X13693422521476","article-title":"Laparoscopic adrenalectomy for nonfamilial adrenal medullary hyperplasia","volume":"17","author":"Mar\u00edn","year":"2013","journal-title":"JSLS"},{"issue":"1","key":"2023081819563595500_dgad121-B8","first-page":"177","article-title":"Adrenal medullary hyperplasia. 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