Cushing's Disease Associated With Empty Sella Turcica: A Case Report

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Abstract

The concurrent presentation of Cushing's disease and empty sella turcica (EST) in the absence of demonstrable pituitary adenoma represents an exceptionally uncommon clinical entity. We report the case of a 68-year-old male patient presenting with systemic arterial hypertension, underlying chronic lymphocytic leukemia, and clinical manifestations consistent with hypercortisolism, who was subsequently referred for specialized endocrinological assessment. Comprehensive endocrine evaluation of adrenocortical function confirmed central hypercortisolism of hypothalamic-pituitary origin, while systematically excluding both adrenal neoplasia and ectopic sources of adrenocorticotropic hormone secretion. Magnetic resonance imaging demonstrated an EST configuration without visualization of any pituitary mass lesion. Therapeutic intervention consisted of stereotactic radiosurgery combined with pharmacological inhibition of steroidogenesis utilizing ketoconazole at 400 mg daily dosing. This regimen successfully normalized urinary free cortisol excretion and restored serum cortisol concentrations to physiological ranges. The patient has maintained ketoconazole, cabergoline and levothyroxine therapy for six months with sustained therapeutic efficacy, demonstrating neither adverse effects nor tachyphylaxis. Normalization of cortisol secretion yielded substantial improvement in blood pressure control and restoration of normal leukocyte parameters, although physical stigmata of chronic hypercortisolism persisted. Serial radiographic surveillance of the pituitary gland throughout the follow-up period has not revealed any adenomatous lesions. This case exemplifies the rare coexistence of EST syndrome and Cushing's disease, highlighting the diagnostic complexities inherent to this association. We conducted a comprehensive literature review to elucidate potential pathophysiological mechanisms underlying this unusual clinical correlation.

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