Severe hepatocytolisis syndrome - a challenge in medical therapy of Cushing’s disease

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ABSTRACT

Introduction Cushing’s disease is a rare endocrine disorder characterized by persistent hypercortisolism due to excessive, autonomous ACTH secretion by a pituitary adenoma. Transsphenoidal adenomectomy is the main therapeutic option in Cushing’s disease. When imaging studies are unable to demonstrate an obvious pituitary mass complete tumor resection is difficult and often encumbered by risk of relapse. Medical therapy is a second-line option or the first-line treatment for patients with high surgical risk, incomplete surgical resection or relapse. Rarely classic clinical manifestations limit the selection of medical therapy for Cushing’s disease. Case presentation We present the case of a male patient with Cushing’s disease complicated with diabetes mellitus and severe hepatocytolisis syndrome with no visible pituitary adenoma on MRI studies. In the absence of technical equipment petrosal sinus sampling could not be performed in order to improve surgery outcome

Conclusion The presence of unbalanced diabetes and severe hepatocytolisis syndrome made it impossible to use steroidogenesis inhibitors (mitotane, ketoconazole, metyrapone, etomidate) or inhibitors of ACTH secretion as pasireotide that represent a new option in achieving control of hypercortisolism

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