ReviewFrontiers in endocrinology2024
Adrenal adenoma secreting 17-hydroxyprogesterone mimicking non-classical 21-hydroxylase deficiency.
Review in Frontiers in endocrinology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Adrenal Venous Sampling Aids in Distinguishing 17-Hydroxyprogesterone Hypersecreting Adrenal Cortical Adenomas from Non-Classical 21-Hydroxylase Deficiency.Diagnostics (Basel, Switzerland) · 2026Article
- Mild Autonomous Cortisol Secretion in Congenital Adrenal Hyperplasia Managed With Mini Back Scope Adrenalectomy.JCEM case reports · 2026Article
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10 authors.
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Abstract
In adrenal steroidogenesis, 17-hydroxyprogesterone (17-OHP) is a substrate for 21-hydroxylase, one of the crucial enzymes of the cortisol and aldosterone biosynthesis pathway. Thus, measurement serum 17-OHP concentration is used when the diagnosis of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is suspected. In the classic 21-hydroxylase deficiency, randomly timed measurements of 17-OHP are generally significantly elevated using different immunoassays. In the non-classic form of CAH (NC-CAH), the activity of 21-hydroxylase is less decreased, therefore the measurements of 17-OHP after ACTH stimulation test are usually required for diagnosis. Nonetheless, elevated 17-OHP concentration may also origin from adrenal tumors or ovarian neoplasms as a result of defects in steroidogenesis with an accumulation of steroids precursors. The presented cases and the literature review draw attention to the occurrence of rare causes of benign adrenal adenomas with steroidogenesis defects which may lead to a misdiagnosis of CAH.
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