Endocrine Abstracts

Cushing’s syndrome is a condition characterized by hypercortisolism (endogenous and exogenous) regardless of its etiology. Hypercortisolism caused by an adrenocorticotropin secreting pituitary adenoma is defined as Cushing’s disease. These tumors represent 10-12% of all pituitary adenomas. ACTH-secreting macroadenomas occur in only 4-23% of all patients with Cushing’s disease. We present the case of 56-year-old female patient with hypercortisolism and stigmata of Cushing’s syndrome. On physical examination we registered "buffalo hump", "facies lunata", hematomas on the skin and central obesity. Laboratory findings verified a disturbed diurnal cortisol rhythm with elevated ACTH values, as well as high cortisol values in low-dose dexamethasone test and partially suppressible values of cortisol in the high-dose dexamethasone test. An NMR of the pituitary gland was performed, verifying a 1.2 cm pituitary macroadenoma. The patient was further referred to a neurosurgeon and neurosurgical treatment was performed (PH: pituitary neuroendocrine tumor PiTNET type of corticotroph tumor, densely granulated subtype). Due to the persistent complaints of the patient, hypokalemia, unregulated hypertension and diabetes, the testing was repeated 3 months following surgery. Along with a disturbed diurnal cortisol rhythm, elevated ACTH values were also maintained. The patient has been referred to a higher reference center and treatment with the steroidogenesis inhibitor metyrapone had been initiated. In the further course, a control pituitary NMR was performed 6 months after the first one, and the rest of the 18 mm adenoma was verified. ACTH values were persistently increasing, and the diurnal rhythm of cortisol was still significantly impaired despite metyrapone therapy. The patient was reoperated on, and immediately postoperatively, elevated ACTH values were registered. The patient was advised to continue therapy with newer generation steroidogenesis inhibitors (osilodrostat), which is not registered in our country, and at this moment she is unable to obtain metyrapone, so cabergoline as only option was introduced. In later course, patient received gamma knife radiosurgery, and reevaluation in order to assess effects will be made. As already stated, Cushing’s disease is a rare condition with many challenges in treatment because in a certain number of cases surgical resection does not provide complete remission. Additionally, the treatment of such a patient in our region is hard due to impossibility of obtaining and prescribing steroidogenesis inhibitors, so the only therapeutic option here in case of failure of cabergoline and radiotherapy is ultimately bilateral adrenalectomy.