{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T04:42:52Z","timestamp":1791434572639,"version":"4.3.3"},"reference-count":29,"publisher":"The Endocrine Society","issue":"8","license":[{"start":{"date-parts":[[2024,2,6]],"date-time":"2024-02-06T00:00:00Z","timestamp":1707177600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/501100007311","name":"Deutsche Kinderkrebsstiftung","doi-asserted-by":"publisher","award":["DKS 2014.06"],"award-info":[{"award-number":["DKS 2014.06"]}],"id":[{"id":"10.13039\/501100007311","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007311","name":"Deutsche Kinderkrebsstiftung","doi-asserted-by":"publisher","award":["DKS 2017.16"],"award-info":[{"award-number":["DKS 2017.16"]}],"id":[{"id":"10.13039\/501100007311","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007311","name":"Deutsche Kinderkrebsstiftung","doi-asserted-by":"publisher","award":["DKS 2021.11"],"award-info":[{"award-number":["DKS 2021.11"]}],"id":[{"id":"10.13039\/501100007311","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,7,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Context<\/jats:title>\n                  <jats:p>Adrenocortical carcinomas are very rare malignancies in childhood associated with poor outcome in advanced disease. Most adrenocortical tumors (ACT) are functional, causing signs and symptoms of adrenal hormone excess. In most studies, endocrine manifestations were reported 4 to 6 months prior to diagnosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>We sought to extend knowledge on endocrine manifestations with regard to age and sex to facilitate early diagnosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We retrospectively analyzed features of adrenal hormone excess in children and adolescents with ACT registered with the GPOH-MET studies between 1997 and 2022. Stage of puberty was defined as prepubertal in females &amp;lt;\u202f8 years of age and males &amp;lt;\u202f9 years.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>By December 2022, 155 patients (110 female, 45 male) with data on endocrine manifestations had been reported. Median age at ACT diagnosis was 4.2 years [0.1-17.8], median interval from first symptoms was 4.2 months [0-90.7]. In 63 girls of prepubertal age, the most frequently reported manifestations were pubarche (68.3%), clitoral hypertrophy (49.2%), and weight gain (31.7%); in 47 pubertal female patients, the most frequent manifestations were excessive pubic hair (46.8%), acne (36.2%), and hypertension (36.2%). Leading symptoms in 34 boys of prepubertal age were pubarche (55.9%), penile growth (47.1%), and acne (32.4%), while in 11 pubertal male patients, leading symptoms were weight gain (45.5%), hypertension (36.4%), excessive pubic hair (27.3%), and cushingoid appearance (27.3%). In pubertal patients, symptoms of androgen excess were mainly unrecognized as part of pubertal development, while symptoms of Cushing syndrome were more frequently apparent.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>The endocrine phenotype induced by pediatric ACT is age- and sex-dependent.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/clinem\/dgae073","type":"journal-article","created":{"date-parts":[[2024,2,6]],"date-time":"2024-02-06T13:28:48Z","timestamp":1707226128000},"page":"2053-2060","source":"Crossref","is-referenced-by-count":7,"title":["The Endocrine Phenotype Induced by Pediatric Adrenocortical Tumors Is Age- and Sex-Dependent"],"prefix":"10.1210","volume":"109","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2672-4045","authenticated-orcid":false,"given":"Marina","family":"Kunstreich","sequence":"first","affiliation":[{"name":"Paediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg , Augsburg 86156 , Germany"},{"name":"Department of Paediatrics, Paediatric Haematology\/Oncology, Otto-von-Guericke-University , Magdeburg 39120 , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Desiree","family":"Dunstheimer","sequence":"additional","affiliation":[{"name":"Paediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg , Augsburg 86156 , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Mier","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Paediatric Haematology\/Oncology, Otto-von-Guericke-University , Magdeburg 39120 , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul-Martin","family":"Holterhus","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Division of Paediatric Endocrinology and Diabetes, Christian-Albrechts-University Kiel & University Hospital Schleswig-Holstein , Kiel 24105 , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan A","family":"Wudy","sequence":"additional","affiliation":[{"name":"Paediatric Endocrinology & Diabetology, Steroid Research & Mass Spectrometry Unit, Centre of Child and Adolescent Medicine, Justus Liebig University , Giessen 35392 , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Huebner","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Paediatric Endocrinology, University Hospital Dresden, Technische Universit\u00e4t Dresden , Dresden 01307 , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1732-1869","authenticated-orcid":false,"given":"Antje","family":"Redlich","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Paediatric Haematology\/Oncology, Otto-von-Guericke-University , Magdeburg 39120 , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4577-0503","authenticated-orcid":false,"given":"Michaela","family":"Kuhlen","sequence":"additional","affiliation":[{"name":"Paediatrics and Adolescent Medicine, Faculty of Medicine, University of Augsburg , Augsburg 86156 , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2024,2,6]]},"reference":[{"issue":"9","key":"2024071216415983700_dgae073-B1","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1016\/j.jpedsurg.2014.03.006","article-title":"Adrenocortical tumors in Italian children: analysis of clinical characteristics and P53 status. 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