Introduction: Cushing syndrome (CS) result from inappropriate exposure to increased non regulated glucocorticoid levels. It remains challenging regarding diagnosis and management. Pituitary ACTH-secreting adenomas account for most of the cases if exogenous and paraneoplasic forms are excluded.
Case report: A 45-year-old women was referred to the endocrine outpatient department because of obesity, muscular weakness, high blood pressure (HBP), diabetes mellitus (DM) and hypercholesterolemia beginning 3 years before. Analytic evaluation evidenced ACTH- dependent hypercortisolism, with no cortisol suppression after dexametasone 1mg overnight. Low-dose dexametasone (0.5 mg quid for 48 h) excluded pseudocushing; high dose dexamethasone (2 mg quid for 48 h) suggested pituitary CS. However no lesion was identified in pituitary RMN and inferior petrous sinus catheterization with simultaneous CRH dynamic testing was inconclusive. Treatment with metyrapone 1500 mg/daily normalized 24-h urinary cortisol excretion and morning cortisol levels, but clinical manifestations persisted with progressive weight gain, worsened diabetes control, increased blood pressure levels and pathologic rib fractures, despite additional drugs. The patient was submitted to bilateral adrenalectomy, which immediately controlled the disease with no further need for antidiabetic or blood pressure drugs and significant weight loss.
Discussion: Even when imaging and functional evaluation are diagnostic, CS treatment may be challenging. Bilateral adrenalectomy is not the first line therapeutic option, but as in this case correction of hypercortisolism more than apparent normalization of cortisol levels may require it. Life-long morbidity is expected regarding glico- and mineralocorticoid reposition, however this is easier to obtain and may be associated with less co-morbidities than sustained hypercortisolism.
20 - 23 May 2017
European Society of Endocrinology