{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T17:54:27Z","timestamp":1774634067743,"version":"3.50.1"},"reference-count":44,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,12,5]],"date-time":"2022-12-05T00:00:00Z","timestamp":1670198400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100006722","name":"Centrum f\u00d6r Klinisk Forskning Dalarna","doi-asserted-by":"publisher","award":["CKFUU-963295"],"award-info":[{"award-number":["CKFUU-963295"]}],"id":[{"id":"10.13039\/501100006722","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006285","name":"Magnus Bergvalls Stiftelse","doi-asserted-by":"publisher","award":["2018-02566, 2019-03149, 2020-03724, 2021-04226"],"award-info":[{"award-number":["2018-02566, 2019-03149, 2020-03724, 2021-04226"]}],"id":[{"id":"10.13039\/501100006285","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Endocrinol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Undiagnosed congenital adrenal hyperplasia (CAH) can cause adrenal incidentalomas, but the frequency is unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>This study aimed to investigate the prevalence of CAH in a population with adrenal incidentalomas and report the clinical characterization.<\/jats:p><\/jats:sec><jats:sec><jats:title>Material and methods<\/jats:title><jats:p>This was a prospective study performed at a regional hospital from 2016 to 2021. Patients with adrenal incidentalomas were investigated with an adrenocorticotropic hormone (ACTH)-stimulation test in addition to hormonal workup. Serum cortisol and 17-hydroxyprogesterone (17OHP) were analyzed. Individuals with a basal or stimulated 17OHP \u226530 nmol\/L were classified as suspicious non-classic CAH, and a <jats:italic>CYP21A2<\/jats:italic>-gene analysis was performed in these subjects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 320 individuals with adrenal incidentalomas were referred to the center, and of these individuals, an ACTH-stimulation test was performed in 222 (median age, 67 (24\u201387) years; 58.6% women; and 11.7% with bilateral lesions). None of the individuals presented a basal 17OHP \u226530 nmol\/L, but there were 8 (3.6%) who did after ACTH stimulation. Four of these subjects (50%) presented bilateral lesions, and the tumor size was larger compared to that of the individuals with a stimulated 17OHP &amp;lt;30 nmol\/L (median, 38 (19\u201366) vs. 19 (11\u201385) mm, p=0.001). A <jats:italic>CYP21A2<\/jats:italic> variation (p.Val282Leu) was detected in one of the eight subjects with a stimulated 17OHP \u226530 nmol\/L, i.e., the patient was a heterozygotic carrier. None of the eight subjects presented with cortisol insufficiency or clinical signs of hyperandrogenism.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The prevalence of non-classic CAH in an adrenal incidentaloma cohort was 3.6% based on stimulated 17OHP and 0% based on gene analysis. CAH should be considered in AI management in selected cases and confirmed by genetic analysis.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fendo.2022.1017303","type":"journal-article","created":{"date-parts":[[2022,12,5]],"date-time":"2022-12-05T05:11:35Z","timestamp":1670217095000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":6,"title":["Congenital adrenal hyperplasia is a very rare cause of adrenal incidentalomas in 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