Endocrine Abstracts

Corticotroph macroadenomas are exceptionally found in Cushing’s disease. Pituitary apoplexy is a rare endocrine emergency condition caused by either hemorrhage or infarction of the pituitary gland. We report a case of a 56-year-old female diagnosed with type 2 diabetes in 2008. One year after, at the periodical follow up, she presented with uncontrolled diabetes (HbA1c=12.26%) and clinical manifestations suggestive for hypercortisolism, therefore insulin therapy was initiated; hormonal profile revealed baseline cortisol=26.4 ng/ml, cortisol level after low-dose dexamethasone suppression test=21.7 ng/ml, ACTH=122 ng/ml, and a 2.4/1.8/2.9 cm pituitary macroadenoma was found. While waiting for surgery appointment, in January 2010 the patient experienced severe headache, nausea, vomiting and diplopia. She was admitted to ICU, diagnosed with pituitary apoplexy and transferred to neurosurgery department where surgery was performed. Clinical improvement of Cushing’s symptoms, diplopia, hypertension and diabetes (HbA1c=6.5% without insulin therapy) was noted. The postoperative pituitary MRI revealed a rest tumor (7/8 mm); slightly increased ACTH levels (70.1 pg/ml) and abnormal 1 mg DXM overnight suppression test (5.33 ng/ml). She received medical treatment with ketoconazole (not tolerated) and cabergoline (2 mg/week); the clinical manifestation of hypercotisolism reappeared and diabetes was poorly controlled. Despite medication, ACTH and cortisol secretion remained elevated and pituitary MRI scan showed tumor progression. Considering the development of the tumor in close proximity of the optic chiasm she was refused for surgery and gamma knife radiation therapy, being lost to follow-up for 2 years. In 2016 she was admitted with severe hepatic cytolysis (ALT=766 U/l, AST=484 U/l), mild cholestatic syndrome, hypercortisolism and uncontrolled diabetes HbA1c=9.6%. The presence of hepatic cytolysis caused postponement of bilateral adrenalectomy. Our patient presented pituitary apoplexy in a corticotroph macroadenoma, with persistence of Cushing’s disease after surgery and no therapy of hypercortisolism at the moment. Bilateral adrenalectomy is probably the best option, after the control of hepatic function.

Keywords: pituitary apoplexy, corticotroph macroadenoma