Abstract
Introduction: Cushing`s syndrome (CS) is caused by prolonged exposure to increased levels of endogenous or exogenous glucocorticoids. Endogenous hypercortisolism is a rare condition which manifests itself with a number of specific morphological features and can lead to hypertension, osteoporosis and diabetes. A number of laboratory analyses and diagnostic procedures are usually required to distinguish between adrenocorticotropic hormone (ACTH)-dependent (pituitary microadenoma, hypothalamus hyperfunction, ectopic secretion of ACTH or corticotropin-releasing hormone) and ACTH-independent (adrenal tumor, macronodular adrenal hyperplasia) etiologies of CS. Materials and methods: A 53-year-old woman is presented with a typical clinical constellation of CS - centripetal obesity, muscle weakness, hypertension, moon face, facial plethora, buffalo hump, purple striae and hirsutism. The laboratory tests displayed increased serum and urine concentrations of cortisol. Computed tomography scan revealed a nodule in the left adrenal gland. Contrast-enhanced MRI showed evidence of pituitary microadenoma and transsphenoidal adenomectomy was performed. Results: High serum and urine cortisol levels persisted postoperatively while ACTH value was within the reference range. The 8-mg dexamethasone suppression test demonstrated a decrease in the morning cortisol levels to 209,37 nmol/l, although not reaching the target cut-off (50 nmol/l). Data for recurrent pathologies in the pituitary gland or change in the nodule size were not detected. Further examination included an invasive diagnostic procedure - bilateral inferior petrosal sinus sampling (BIPSS), which indicated elevated levels of ACTH in the right inferior petrosal sinus - 138 pg/ml (compared to 30,2 pg/ml in the serum). BIPSS proved the central genesis of CS. As a therapeutic opportunity, palliative adrenalectomy was discussed. Conclusion: CS often presents a clinical and diagnostic dilemma. BIPSS is an invasive and hard to perform specialized procedure. Although being useful for establishing the source of ACTH production, it has to be performed only in high technology centers.
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