Adrenal gland
Vasiliki Ioanna Mitravela 1 , Maria Giannakou 1 , Efstratios Kardalas 1 , Aikaterini Lavrentaki 1 , Panagiotis Mouchtouris 1 , Dimitra Baikousi 1 , Efthimia Botoula 1 , George Kyriakopoulos 2 , Dimitra Argyro Vassiliadi 1 & Stylianos Tsagarakis 1
1Department of Endocrinology, Diabetes and Metabolism, National Expertise Centre for Rare Diseases, Evangelismos General Hospital of Athens, Athens; [email protected]; 2Department of Pathology, Evangelismos General Hospital of Athens, Athens
Background: In patients with adrenal nodules increased 17-OH Progesterone (17-OHP) does not necessarily indicate congenital adrenal hyperplasia (CAH) since adrenocortical tumors may harbor defects in steroidogenesis resulting in high steroid precursors, either basally or, usually, after Synacthen stimulation. Secretion of precursors has been associated with malignancy but it can also be present in benign adenomas.
Case Presentation: A 62-year-old lady with hypertension and dyslipidemia was referred for bilateral incidentalomas (a left mass gradually increasing over 14 years from 2.3 to 4.7 cm and a stable 1.4 cm right adenoma known since 2016). Past medical and gynaecological history were unremarkable. On clinical examination mild moon face and central myopathy were noted, BMI was 28.4 kg/m2. Hormonal investigation showed mild autonomous cortisol excess (cortisol post 1 mg dexamethasone 2 μg/dl) ACTH 16.9 pg/ml and UFC 61 μg/24h. Basal levels of 17-OHP were high but varied widely on three occasions (50.6, 29.3 & 12.5 ng/ml) whereas androgens were low (testosterone 6 ng/dl, androstenedione 0.9 ng/ml and DHEA-S<15 μg/dl). Stimulated levels of 17-OHP were 49.5ng/ml. The patient underwent left adrenalectomy (5 cm, Ki-67:1%, Weiss: 0/9), the levels of 17-OHP normalized (2 ng/ml) whereas androgens remained suppressed. She did not develop adrenal insufficiency (morning cortisol 12.1 μg/dl, ACTH 46.8 pg/ml).
Conclusions: High 17-OHP level related to CAH due to CYP21A2 or CYP11B1 deficiency was not likely based on low androgens and the lack of past medical history compatible with a genetic steroidogenesis defect. Given the biochemical features suggestive of combined CYP21A2 and CYP17A1 impairment, we postulate the adrenal tumor secreted 17-OHP due to steroidogenic enzymatic defect probably secondary to the adenomatous proliferation but CYP staining or mRNA sequencing was not performed.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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