Size of Cortisol-Secreting Adrenal Adenoma, Clinical Symptoms and Severity of Hypercortisolism: Case Study of Two Patients

Presentation Number: SUN-0788
Date of Presentation: June 22nd, 2014

Dinesh Edem*1, Esther Irina Krug2, Ivana Milojevic3 and Amitoj Gill3
1Sinai Hospital of Baltimore, Baltimore, MD, 2Johns Hopkins University/ Sinai Hosp of Baltimore, Baltimore, MD, 3Johns Hopkins University/Sinai Hospital of Baltimore, Baltimore, MD

Abstract

Introduction: Cushings Syndrome (CS) is a rare disorder caused by prolonged exposure to endogenous or exogenous glucocorticoids. Incidence of CS ranges from 0.7 to 2.4 per million(1). Symptoms of CS including obesity, diabetes, hypertension, bone loss and depression are common in the population, making the diagnosis of CS extremely difficult. We present a case study of 2 patients with CS due to adrenal adenoma and analysis of correlation between severity of symptoms, tumor size and degree of hypercortisolism.

Case 1: A 56 year old woman with T2DM, hypertension and hirsutism returns for follow-up with complaints of gradual weight gain, increasing abdominal girth and leg cramps. She has family history of T2DM, osteoporosis and diabetes. Physical exam is notable for abdominal obesity,plethora, round facies, and supraclavicular fat pads. Serial 11 PM salivary cortisol ranged between 4 - 7.1 ng/mL(n <4.3), an overnight DST revealed a cortisol of 7.8 mcg/dL(n < 5), random ACTH level was < 0.5 pg/mL(6-50) with corresponding cortisol of 18.2 mcg/dL. CT scan of the abdomen revealed a 5.5 X 4.7 cm right adrenal adenoma which was subsequently resected. Patient had an uneventful postoperative course with no evidence of adrenal insufficiency. In follow-up her weight decreased by 10 lbs and diabetes improved. Repeat testing a year later showed ACTH of 28, and DST showed cortisol of 0.5.

Case 2 : A 74 year old woman with postmenopausal osteoporosis, hypothyroidism, T2DM on metformin returns to clinic with a 3 month onset of increasing abdominal girth, weight gain, worsening fatigue, proximal  muscle weakness of her extremities and poor blood pressure control. DXA revealed significant bone loss despite ongoing treatment with Denosumab. Exam revealed normal BMI, increased abdominal girdth and muscle weakness, but no moon facies, plethora or striae. Serial 11 PM salivary cortisol ranged from 7.2 to 13.6 ng/mL(n <4.3), UFC of 162 mcg/24h (0-50), ACTH level < 5 pg/mL with corresponding cortisol level of 19mcg/dL . CT of the abdomen revealed a 4.5 X 2.1 cm left adrenal mass. Patient subsequently underwent a resection of a benign left adrenal cortical adenoma. Postoperatively on day 2, patient developed symptoms of adrenal insufficiency.  Hydrocortisone therapy resulted in resolution of symptoms. Her AI  gradually improved after several months of glucocorticoid replacement.

Discussion: These cases illustrate that in patients with CS due to cortisol secreting adrenal adenoma, clinical symptoms and tumor size may not correlate with severity of hypercortisolism and risk of post-operative adrenal suppression. Our patients had adrenal adenomas of almost the same size, but had varied clinical and biochemical picture. Risk of postoperative adrenal insufficiency appears to correlate with the preoperative severity of hypercortisolism.

 

Nothing to Disclose: DE, EIK, IM, AG