MON-469 - Behind The Mask: Diagnosing Cushing Syndrome With An Atypical Presentation

Endogenous Cushing syndrome is a clinical disorder caused by chronic exposure to excessive levels of cortisol produced within the body, rather than from exogenous (iatrogenic) glucocorticoid administration. The endogenous cause of cushing’s syndrome include adrenal adenomas or carcinomas that are ACTH-independent.Endogenous Cushing's syndrome is rare, with an estimated incidence of 2–8 cases per million people annually.We present a case of diagnosing Cushing Syndrome with atypical features.

A 39 year old Caucasian Female with History of ADHD, HTN, Overweight (BMI 28.3 ) presented to Endocrine Clinic with somatic symptoms like arthralgias, headaches and myalgias . Her workup including infectious, cardiac, neurology, autoimmune, hematologic was negative.
.Lab Values showed hypercortisolism with Random Cortisol 15.43mcg/dl (8.7-22.4), ACTH < 1.5pg/ml (7.2-63.3). Cortisol after 1mg Dexamethasone Test 14.98mcg/dl( < 1.8), Midnight Salivary Cortisol 1st Set 0.18ug/dl, 2nd set 0.139ug/dl ( < 0.010-0.090) 24H Urinary Cortisol 1st set 53ug/24h ,2nd set 68ug/24h (6-42). Further imaging was ordered to diagnose the cause of hypercortisolism and CT Abdomen with adrenal Protocol was ordered that showed a 2.3 cm enhancing right adrenal gland mass adenoma and left adrenal gland is normal..Work up done for hyperaldosterosnism, hyperandrogenemia, pheochromocytoma was negative.On account of hypercortisolism and CT findings of an adrenal mass patient was referred to Endocrine Surgery .This patient had a Right Retroperitoneal Adrenelectomy and postoperatively patient was kept on Hydrocortisone .Serial monitoring for Cortisol, ACTH was done. Cosyntrophin stimulation test was done to check for the response of cortisol and cortisol adequately increased, after 5 months postoperatively hydrocortisone was stopped as HPA recovered. Pathology came back positive for Adrenal Cortical Adenoma. (2.4cm).At follow up, the patient demonstrated marked clinical improvement in presenting symptoms of somatic complaints

Cushing's syndrome with atypical features refers to presentation of endogenous hypercortisolism that do not display the classic constellation of cushingoid signs (such as facial plethora, purple striae, proximal muscle weakness, and easy bruising), but instead manifest with subtler, non-specific, or less overt symptoms Hence It is important to diagnose Cushing syndrome when it presents with atypical features because untreated hypercortisolism, even when mild or atypical, is associated with significant morbidity and increased mortality due to metabolic, cardiovascular, musculoskeletal, and neuropsychiatric complications..The Endocrine Society specifically recommends screening for Cushing syndrome in patients with unusual or progressive features, or in those with adrenal incidentalomas, to avoid the consequences of unrecognized disease

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