Session: SUN 29-49-Congenital Adrenal Hyperplasia & Ectopic Cushing's
Poster Board SUN-36
Clinical case: A 29-yo G4A3 woman was found at 25 weeks of pregnancy to have a 3.4 X 3.1 cm right adrenal lesion during evaluation of nephrolithiasis. She had signs of CS, mild hypertension and gestational diabetes requiring 100 U of insulin/d by 34 weeks of gestation. Investigation revealed loss of diurnal plasma cortisol levels and elevated urinary free cortisol (UFC) of 787 nmol/d at 25 weeks and 4338 nmol/d at 31 weeks of gestation (N: 98-500). Corresponding plasma ACTH levels were not suppressed at 4 and 6 pmol/L (N: 2-11).
One month post-partum her 24 h UFC had normalized (107 nmol/d, N <220) with corresponding suppressed ACTH of 1.1 pmol/L. Plasma cortisol did not suppress during a 4 mg IV dexamethasone test (basal: 304 nmol/L; next morning cortisol: 365 nmol/L). CT scan re-demonstrated a 2.9X3.7 cm adrenal lesion with density of 20 HU. Right adrenalectomy was performed 7 months post-partum; pathology demonstrated an adrenocortical adenoma (Weiss score: 0) and atrophy of adjacent normal cortex. She remains glucocorticoid-dependent six months post-operatively.
Results: In vivo screening tests performed 6 weeks post-partum to identify possible aberrant adrenal receptors (1,2) showed no cortisol response (including iv LHRH 100 µg and hLH 300 U) except following administration of 250 ug cosyntropin iv (basal: 399 to 30 min peak of 848 nmol/L). Quantitative RT-PCR analysis of the adenoma revealed levels of MC2 receptor mRNA levels similar to normal control adrenals, while LHCG receptor mRNA was almost undetectable.
Conclusions: This study suggests that cortisol production by some adrenal adenomas can be enhanced during pregnancy by placental derived ACTH which is not completely suppressed by the cortisol overproduction.
Disclosure: JMM: , Serono, , Novartis Pharmaceuticals. AL: Investigator, Novartis Pharmaceuticals, Consultant, Novartis Pharmaceuticals, Editor, Up To Date. Nothing to Disclose: SO, RAB
*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm
See more of: Abstracts - Orals, Featured Poster Presentations, and Posters