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Review of Cushing Syndrome

Many patients with Cushing syndrome require medications, radiation, or bilateral adrenalectomy if first-line removal of the causative tumor is not sufficient to relieve symptoms, according to the authors of a review article. Thought to be underdiagnosed, the condition is reported in 2 to 8 people per million annually, with a usual age of onset in the 30- to 49-year-old range.

“Cushing syndrome characteristically presents with skin changes such as facial plethora, easy bruising, and purple striae and with metabolic manifestations such as hyperglycemia, hypertension, and excess fat deposition in the face, back of the neck, and visceral organs,” the authors write. “Cushing disease, in which corticotropin excess is produced by a benign pituitary tumor, occurs in approximately 60% to 70% of patients with Cushing syndrome due to endogenous cortisol production.”

The condition can also be caused by ectopic sources of corticotropin in the lung, mediastinum, pancreas, and medullary thyroid gland, or by cortisol-producing adenomas in the adrenal cortex. Diagnosis involves ruling out exogenous steroid use and tests that distinguish between “adrenal causes of hypercortisolism (suppressed corticotropin) and corticotropin-dependent forms of hypercortisolism (midnormal to elevated corticotropin levels),” the authors write. “Management of Cushing syndrome begins with surgery to remove the source of excess endogenous cortisol production followed by medication that includes adrenal steroidogenesis inhibitors, pituitary-targeted drugs, or glucocorticoid receptor blockers. For patients not responsive to surgery and medication, radiation therapy and bilateral adrenalectomy may be appropriate.”

Source: JAMA