Abstract


Introduction: 9%  of unilateral adrenal adenomas cause Cushing’s syndrome (CS) due to cortisol (C) excess. Depending on the amounts of glucocorticoids (GC) secreted by the tumor, the clinical spectrum ranges from altered diurnal cortisol rhythm to atrophy of contralateral adrenal with lasting adrenal insufficiency (AI) after unilateral adrenalectomy (AE). Patients with suppressed plasma ACTH generally have AI after surgery and  need GC replacement until recovery of the hypothalamic–pituitary–adrenal (HPA) axis ( 6–18 months after surgery). view more

Introduction: 9%  of unilateral adrenal adenomas cause Cushing’s syndrome (CS) due to cortisol (C) excess. Depending on the amounts of glucocorticoids (GC) secreted by the tumor, the clinical spectrum ranges from altered diurnal cortisol rhythm to atrophy of contralateral adrenal with lasting adrenal insufficiency (AI) after unilateral adrenalectomy (AE). Patients with suppressed plasma ACTH generally have AI after surgery and  need GC replacement until recovery of the hypothalamic–pituitary–adrenal (HPA) axis ( 6–18 months after surgery). Mifepristone (Mif), a GC and progesterone-receptor antagonist, is approved in US for control of hyperglycemia due to hypercortisolism in patients with CS.  Mif binds to human GC receptors with an affinity 3 to 4 times higher than that of dexamethasone and about 18 times higher than that of C. Mif affects central actions of C (negative feedback on CRH/ACTH secretion) and its peripheral actions. Due to rapid onset of action and long half-life Mif may allow preoperative restoration of HPA axis and reversal of cortical atrophy in unilateral adrenal adrenal CS.  
Case:The patient is a 56 year old woman  with history of hirsutism, adult acne, obesity, irregular menstrual cycles, male pattern balding throughout adult life who presented with worsening hyperandrogenism (HA), plethora and labile hypertension during menopausal transition, confirmed by physical exam.. She had unrevealing screening for CS and adrenal HA 5 years previously. Labs revealed impaired glucose tolerance (HbA1c - 6.0%), cholesterol 345 mg/dL, triglycerides 486 mg/dL, total testosterone (TT) 153 ng/dL (3-40), free testosterone 25.8  pg/mL (0.0-4.2), DHEA-S 145.4 ug/dL, FSH 28.9 mIU/mL, LH – 19.2 mIU/ml, aldosterone -8.4 ng/dL, plasma renin activity – 0.28 ng/mL/hr. Dexamethasone 1 mg suppression test revealed 08:00 serum C - 7.6 ug/dL . Random ACTH was 3.8 pg/mL, C - 11.8 ug/dL. MRI of the abdomen revealed 2.5 x 3.1 x 2.6 cm right adrenal mass. After administration of Leuprolide Depot, 7.5 mg IM, TT – 89 pg/mL, FT – 10.3 pg/mL, DHEA-S - 88.2 ug/dL, suggesting significant adrenal androgen production.  In preparation for AE Mif, 300 mg po qod was initiated. After 30 days repeat ACTH – 28.3 pg/mL. She had improvement in mood, fatigue, BP, 3 kg weight loss after 2 months. Last dose of MIf was given 48 h before  AE after 10 weeks of treatment. After laparoscopic AE she had no symptoms of AI and no GC replacement. In 48 h after AE, ACTH – 20.3 pg/mL, C – 19.0 ug/dL. In 30 days, ACTH – 31.1 pg/mL, C – 6.8 ug/dL, TT – 19 ng/dL, DHEA-S – 17.7 ug/dL. There were no symptoms of AI. Marked reduction in TT and DHEA-S after AE was consistent with androgen co-secretion by the tumor.                                                                                           
Discussion: This case suggests efficacy and safety of Mif in restoration of HPA axis in patients with CS due to unilateral adrenal adenoma in preparation to AE. Serial ACTH measurements appear to reliably guide Mif dosing and help prevent AI due to excessive GC receptor blockade.

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