{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T04:24:34Z","timestamp":1790915074780,"version":"4.1.0"},"reference-count":43,"publisher":"Georg Thieme Verlag KG","issue":"10","funder":[{"name":"Korean government","award":["NRF2021R1F1A104593011"],"award-info":[{"award-number":["NRF2021R1F1A104593011"]}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"crossref","award":["F2021R1F1A104593011"],"award-info":[{"award-number":["F2021R1F1A104593011"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Exp Clin Endocrinol Diabetes"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n          Objective Adrenal tumors are generally rare in children and can be a part\n                    of familial cancer syndrome. This research was conducted to examine the clinical\n                    outcomes, histopathological results, and genetic etiologies of adrenal tumors in\n                    children and adolescents.<\/jats:p><jats:p>\n          Methods Thirty-one children and adolescents with adrenal tumors were\n                    included. Data on clinical outcomes and endocrine and radiologic results were\n                    retrospectively analyzed. Molecular analysis was conducted in select patients\n                    according to their phenotype and family history.<\/jats:p><jats:p>\n          Results The median age at diagnosis was 7.9 years (range: 0.8\u221217.8\n                    years) with 5.1\u00b11.8\u2009cm of maximum tumor diameter. Adrenal\n                    adenoma (n=7), carcinoma (n=5), borderline (n=2),\n                    isolated micronodular adrenocortical disease (n=2), pheochromocytoma\n                    (n=8), paraganglioma (n=3), and ganglioneuroma (n=4) are\n                    all pathological diagnoses. The most common presenting symptom was excess\n                    production of adrenocortical hormones (n=15), including virilization and\n                    Cushing syndrome. Non-functioning adrenocortical tumors were found in a patient\n                    with congenital adrenal hyperplasia. Genetic etiologies were identified in\n                        TP53 (n=5), VHL (n=4), and PRKACA\n                    (n=1). Patients with mutations in TP53 were young\n                    (1.5\u00b10.5 years) and had large masses (6.1\u00b12.3\u2009cm).<\/jats:p><jats:p>\n          Conclusions This study describes clinical outcomes and the pathological\n                    spectrum of adrenal tumors in children and adolescents. Adrenocortical tumors\n                    mostly presented with an excess of the adrenocortical hormone. Patients with\n                    genetic defects presented at a young age and large size of tumors, necessitating\n                    genetic testing in patients at a young age.<\/jats:p>","DOI":"10.1055\/a-2127-9292","type":"journal-article","created":{"date-parts":[[2023,7,12]],"date-time":"2023-07-12T22:51:11Z","timestamp":1689202271000},"page":"515-522","source":"Crossref","is-referenced-by-count":8,"title":["Clinical Characteristics and Long-Term Outcomes of Adrenal Tumors in\n                    Children and Adolescents"],"prefix":"10.1055","volume":"131","author":[{"given":"Ja Hye","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Asan Medical Center, University of Ulsan\n                    College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yunha","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Asan Medical Center, University of Ulsan\n                    College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Soojin","family":"Hwang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Asan Medical Center, University of Ulsan\n                    College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji-Hee","family":"Yoon","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Asan Medical Center, University of Ulsan\n                    College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gu-Hwan","family":"Kim","sequence":"additional","affiliation":[{"name":"Medical Genetics Center, Asan Medical Center, University of Ulsan\n                    College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han-Wook","family":"Yoo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Asan Medical Center, University of Ulsan\n                    College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin-Ho","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Asan Medical Center, University of Ulsan\n                    College of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2023,9,6]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.mayocpiqo.2017.11.002","article-title":"Clinical, biochemical, and radiological characteristics of a single-center\n                    retrospective cohort of 705 large adrenal tumors","volume":"2","author":"N M I\u00f1iguez-Ariza","year":"2017","journal-title":"Mayo Clinic Proc Innov Qual Outcomes"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"894","DOI":"10.1016\/S2213-8587(20)30314-4","article-title":"Epidemiology of adrenal tumours in Olmsted County, Minnesota, USA: A\n                    population-based cohort study","volume":"8","author":"A Ebbehoj","year":"2020","journal-title":"Lancet Diabetes Endocrinol"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1210\/er.2002-0031","article-title":"The clinically inapparent adrenal mass: Update in diagnosis and management","volume":"25","author":"G Mansmann","year":"2004","journal-title":"Endocr Rev"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"101448","DOI":"10.1016\/j.beem.2020.101448","article-title":"Pediatric adrenocortical tumours","volume":"34","author":"E M Pinto","year":"2020","journal-title":"Baillieres Best Pract Res Clin Endocrinol Metab"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1007\/s12022-022-09710-8","article-title":"Overview of the 2022 WHO classification of adrenal cortical tumors","volume":"33","author":"O Mete","year":"2022","journal-title":"Endocr Pathol"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1007\/s12672-011-0088-0","article-title":"The Weiss score and beyond\u2014histopathology for adrenocortical\n                    carcinoma","volume":"2","author":"M Papotti","year":"2011","journal-title":"Horm Cancer"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.mce.2011.12.008","article-title":"Association of adrenocortical carcinoma with familial cancer susceptibility\n                    syndromes","volume":"351","author":"T Else","year":"2012","journal-title":"Mol Cell Endocrinol"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"23","DOI":"10.3389\/fendo.2015.00023","article-title":"Pediatric adrenocortical tumors: What they can tell us on adrenal development\n                    and comparison with adult adrenal tumors","volume":"6","author":"E Lalli","year":"2015","journal-title":"Front Endocrinol (Lausanne)"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1200\/JCO.2004.08.085","article-title":"Clinical and outcome characteristics of children with adrenocortical tumors: A\n                    report from the international pediatric adrenocortical tumor registry","volume":"22","author":"E Michalkiewicz","year":"2004","journal-title":"J Clin Oncol"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1159\/000488855","article-title":"Adrenocortical carcinoma in children: A clinicopathological analysis of 41\n                    patients at the Mayo Clinic from 1950 to 2017","volume":"90","author":"N Gupta","year":"2018","journal-title":"Horm Res Paediatr"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"155","DOI":"10.3389\/fped.2017.00155","article-title":"Review of pediatric pheochromocytoma and paraganglioma","volume":"5","author":"R Bholah","year":"2017","journal-title":"Front Pediatr"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1530\/ERC-13-0415","article-title":"Long-term prognosis of patients with pediatric pheochromocytoma","volume":"21","author":"B Bausch","year":"2014","journal-title":"Endocr Relat Cancer"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1007\/s12022-017-9484-5","article-title":"Update on adrenal tumours in 2017 World Health Organization (WHO) of endocrine\n                    tumours","volume":"28","author":"AK Lam","year":"2017","journal-title":"Endocr Pathol"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1111\/cen.14460","article-title":"Presentation and outcomes of adrenal ganglioneuromas: A cohort study and a\n                    systematic review of literature","volume":"95","author":"K N Dages","year":"2021","journal-title":"Clin Endocrinol (Oxf)"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"610746","DOI":"10.3389\/fendo.2020.610746","article-title":"Clinical presentation and treatment outcomes of children and adolescents with\n                    pheochromocytoma and paraganglioma in a single center in Korea","volume":"11","author":"H Park","year":"2021","journal-title":"Front Endocrinol (Lausanne)"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"246","DOI":"10.4174\/jkss.2012.82.4.246","article-title":"Adrenocortical tumors in children 18 years old and younger","volume":"82","author":"M J Cho","year":"2012","journal-title":"J Korean Surg Soc"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"136","DOI":"10.6065\/apem.2016.21.3.136","article-title":"Etiology and therapeutic outcomes of children with gonadotropin-independent\n                    precocious puberty","volume":"21","author":"E Kang","year":"2016","journal-title":"Ann Pediatr Endocrinol Metab"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"315","DOI":"10.6065\/apem.2142100.050","article-title":"Adrenocortical carcinoma and a sporadic MEN1 mutation in a 3-year-old girl: A\n                    case report","volume":"27","author":"S E Kim","year":"2022","journal-title":"Ann Pediatr Endocrinol Metab"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"47","DOI":"10.6065\/apem.2016.21.1.47","article-title":"Multiple osteoblastomas in a child with Cushing syndrome due to bilateral\n                    adrenal micronodular hyperplasias","volume":"21","author":"H W Yu","year":"2016","journal-title":"Ann Pediatr Endocrinol Metab"},{"key":"ref20","first-page":"9","article-title":"Parliament2: Accurate structural variant calling at scale","author":"S Zarate","year":"2020","journal-title":"Gigascience"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"3572","DOI":"10.1093\/bioinformatics\/bty304","article-title":"AnnotSV: An integrated tool for structural variations annotation","volume":"34","author":"V Geoffroy","year":"2018","journal-title":"Bioinformatics"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/gim.2015.30","article-title":"Standards and guidelines for the interpretation of sequence variants: A joint\n                    consensus recommendation of the American College of Medical Genetics and\n                    Genomics and the Association for Molecular Pathology","volume":"17","author":"S Richards","year":"2015","journal-title":"Genet Med"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1016\/j.ejca.2009.12.007","article-title":"The European Network for the study of adrenal tumors staging system is\n                    prognostically superior to the international union against cancer-staging\n                    system: A North American validation","volume":"46","author":"G Lughezzani","year":"2010","journal-title":"Eur J Cancer"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1200\/JCO.2013.52.6863","article-title":"Prevalence and functional consequence of TP53 mutations in pediatric\n                    adrenocortical carcinoma: A Children\u2019s Oncology Group Study","volume":"33","author":"J D Wasserman","year":"2015","journal-title":"J Clin Oncol"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1530\/EJE-14-1154","article-title":"Germline PRKACA amplification causes variable phenotypes that may depend on the\n                    extent of the genomic defect: Molecular mechanisms and clinical\n                    presentations","volume":"172","author":"M B Lodish","year":"2015","journal-title":"Eur J Endocrinol"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"31128","DOI":"10.1038\/srep31128","article-title":"VHLdb: A database of von Hippel-Lindau protein interactors and mutations","volume":"6","author":"F Tabaro","year":"2016","journal-title":"Sci Rep"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1056\/NEJMcp065470","article-title":"The incidentally discovered adrenal mass","volume":"356","author":"W F Young","year":"2007","journal-title":"N Engl J Med"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1210\/er.2017-00189","article-title":"Genetic causes of functional adrenocortical adenomas","volume":"38","author":"M-C Zennaro","year":"2017","journal-title":"Endocr Rev"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"e635","DOI":"10.1097\/MPH.0000000000001982","article-title":"Clinical outcomes of children with adrenocortical carcinoma in the context of\n                    germline TP53 status","volume":"43","author":"CT A Brenna","year":"2021","journal-title":"J Pediatr Hematol Oncol"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1210\/er.2010-0001","article-title":"Development and function of the human fetal adrenal cortex: A key component in\n                    the feto-placental unit","volume":"32","author":"H Ishimoto","year":"2011","journal-title":"Endocr Rev"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.mce.2011.09.010","article-title":"Towards an understanding of the role of p53 in adrenocortical\n                    carcinogenesis","volume":"351","author":"J D Wasserman","year":"2012","journal-title":"Mol Cell Endocrinol"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"5065","DOI":"10.1210\/jc.2019-00547","article-title":"Glucocorticoid activity of adrenal steroid precursors in untreated patients with\n                    congenital adrenal hyperplasia","volume":"104","author":"M Engels","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"1351","DOI":"10.4158\/EP-2020-0058","article-title":"Prevalence and characteristics of adrenal tumors and myelolipomas in congenital\n                    adrenal hyperplasia: A systematic review and meta-analysis","volume":"26","author":"I Nermoen","year":"2020","journal-title":"Endocr Pract"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"4925","DOI":"10.1210\/jc.2010-0803","article-title":"Improved survival in patients with stage II adrenocortical carcinoma followed up\n                    prospectively by specialized centers","volume":"95","author":"M Fassnacht","year":"2010","journal-title":"J Clin Endocrinol Metab"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"287","DOI":"10.3892\/etm.2022.11216","article-title":"Adrenocortical carcinoma: Pediatric aspects (Review)","volume":"23","author":"F Sandru","year":"2022","journal-title":"Exp Ther Med"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"3956","DOI":"10.1200\/JCO.2017.74.2460","article-title":"Identification of clinical and biologic correlates associated with outcome in\n                    children with adrenocortical tumors without germline TP53 mutations: A St Jude\n                    Adrenocortical Tumor Registry and Children's Oncology Group Study","volume":"35","author":"E M Pinto","year":"2017","journal-title":"J Clin Oncol"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"913253","DOI":"10.3389\/fendo.2022.913253","article-title":"Cushing\u00b4s syndrome due to bilateral adrenal cortical disease: bilateral\n                    macronodular adrenal cortical disease and bilateral micronodular adrenal\n                    cortical disease","volume":"13","author":"M Araujo-Castro","year":"2022","journal-title":"Front Endocrinol (Lausanne)"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"794","DOI":"10.1038\/ng1809","article-title":"A genome-wide scan identifies mutations in the gene encoding phosphodiesterase\n                    11A4 (PDE11A) in individuals with adrenocortical hyperplasia","volume":"38","author":"A Horvath","year":"2006","journal-title":"Nat Genet"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"750","DOI":"10.1056\/NEJMc0706182","article-title":"Mutation in PDE8B, a cyclic AMP\u2013specific phosphodiesterase in adrenal\n                    hyperplasia","volume":"358","author":"A Horvath","year":"2008","journal-title":"N Engl J Med"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"R109","DOI":"10.1530\/EJE-13-0882","article-title":"Diagnosis of endocrine disease: Biochemical diagnosis of phaeochromocytoma and\n                    paraganglioma","volume":"170","author":"A van Berkel","year":"2014","journal-title":"Eur J Endocrinol"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"1109","DOI":"10.1542\/peds.2005-2299","article-title":"Pheochromocytoma and paraganglioma in children: A review of medical and surgical\n                    management at a tertiary care center","volume":"118","author":"T H Pham","year":"2006","journal-title":"Pediatrics"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"1476","DOI":"10.1016\/j.annonc.2020.08.2099","article-title":"Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO-EURACAN cinical\n                    practice guidelines for diagnosis, treatment and follow-up","volume":"31","author":"M Fassnacht","year":"2020","journal-title":"Ann Oncol"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"2942","DOI":"10.3390\/cancers14122942","article-title":"Postoperative recurrences in patients operated for pheochromocytomas and\n                    paragangliomas: New data supporting lifelong surveillance","volume":"14","author":"S Parisien-La Salle","year":"2022","journal-title":"Cancers (Basel)"}],"container-title":["Experimental and Clinical Endocrinology &amp; Diabetes"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2127-9292.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,6]],"date-time":"2023-10-06T23:50:48Z","timestamp":1696636248000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2127-9292"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7,12]]},"references-count":43,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2023,10,6]]},"published-print":{"date-parts":[[2023,10]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2127-9292","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0947-7349","1439-3646"],"issn-type":[{"value":"0947-7349","type":"print"},{"value":"1439-3646","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,7,12]]},"published":{"date-parts":[[2023,7,12]]}}}