MON-181 - A Case of Sky-High Dehydroepiandrosterone Sulfate and Absence of Adrenal Tumors in A 19 Year-Old Female with Polycystic Ovary Syndrome

Background: Dehydroepiandrosterone sulfate (DHEAS) is a major steroid hormone of the adrenal glands and is an intermediate in the synthesis of testosterone and estrogen. Very high levels of DHEAS are usually indicative of adrenal tumors and less commonly with non-classical congenital adrenal hyperplasia. While elevated DHEAS can also be seen in polycystic ovarian syndrome (PCOS), typically levels are only mildly elevated. Here we present the case of a woman with very high DHEAS in the absence of adrenal tumors with presumed PCOS.
Clinical

Case: A 19-year-old woman with a history of obesity presented for evaluation of oligomenorrhea for 5 years and high testosterone. She endorsed hirsutism (chin and neck) and acne. Follow up laboratory testing showed a very elevated DHEAS of 842 µg/dL (RR 65.1-368), low estradiol 15.7 pg/mL (RR 30.0-100.0 Pre-menopausal, early follicular) and an elevated IGF-1 of 406 ng/mL (RR 85 – 370). Computed tomography (CT) imaging of the adrenal gland showed no adrenal nodules. Pelvic ultrasound showed no ovarian masses. A 5-day dexamethasone suppression test (DAST) resulted in a total suppression of cortisol (14.8 to 0.3 µg/dL) and an approximately 77% suppression of DHEAS (1105 to 258 µg/dL). Free testosterone was not suppressed (16 pg/mL to 12 pg/mL), indicating an ovarian source. Oral glucose tolerance test (OGTT) showed appropriate suppression of growth hormone (GH) to 0.2 ng/mL at 2 hrs post glucose consumption making acromegaly unlikely. ACTH Stimulation test with 250 µg Cosyntropin resulted in a proper stimulation of cortisol to 24.5 µg/dL at 30 min, and 27.1µg/dL at 60 min, a rise in DHEA from 6.8 ng/mL (baseline), to 17 ng/mL at 30 min, and 22 ng/mL at 60 min, and increase of Androstenedione 303 ng/dL at 30 min, 273 ng/dL at 60 min. 17-OH Pregnenolone increased from 516 ng/dL (baseline) to 1960 ng/dL (peak) after Cosyntropin, however levels < 4500 ng/dL did not indicate a 3-Beta-hydroxysteroid dehydrogenase (3β-HSD) deficiency. Peak 17-OH Progesterone was 301 ng/dL following Cosyntropin, making 12-hydroxylase deficiency unlikely. In summary, mildly elevated stimulated levels of 17-OH Pregnenolone and DHEA along with lack of suppression of testosterone on DAST in a young woman with obesity were characteristic of adrenal hyperandrogenism in PCOS. The patient was started on combination oral contraceptives with improvement in menstrual regularity.

Conclusion: This case of oligomenorrhea in young adult female underscores the clinical importance of assessing dehydroepiandrosterone sulfate (DHEAS) levels, even in the absence of an adrenal tumor. Routine evaluation of DHEAS may aid in the early identification of hyperandrogenic etiologies affecting reproductive health in adolescent females. Early recognition and timely intervention are critical in mitigating the reproductive and metabolic consequences associated with elevated DHEAS and hyperandrogenism.

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