This report presents a 44-year-old woman with ectopic ACTH-secreting pituitary adenoma located in the supradiaphragmal region. Patient presented with a classical clinical features of long lasting Cushings syndrome. She had right leg amputation because of peripheral macrovascular atherosclerosis, severe hypertension, non-regulated diabetes mellitus, hypokalemia and osteoporosis. Baseline tests were suggestive of ACTH-dependent Cushings syndrome. Magnetic resonance imaging revealed hypodensic lesion in the right side of the pituitary 6 mm in diameter, and an oval and well-circumscribed supradiaphragmal tumour 34 mm in diameter in the ventral and the left side of the stalk. Both lesions enhaced by gadolinium. Transphenoidal resection of suspected eutopic pituitary adenoma was performed. Histology revealed normal pituitary tissue, and the cortisol level remained high after the operation. After the first operation, supraorbital key-hole resection of suspected ectopic pituitary adenoma was performed. Histology and imunohistology confirmed ACTH producing adenoma. After the second operation plasma cortisol level became low. Postoperative period was complicated by repeating urinary tract infections and infection of left thumb. She was treated with hydrocortisone supplementation and antibiotics. Ten days after discharge from the hospital she was admitted to the local hospital in severe state and she died after few hours. This is the first report in the literature of the patient with ACTH dependent Cushings syndrome caused by supradiaphragmatic ectopic ACTH-secreting adenoma with positive magnetic resonance imaging for eutopic and ectopic supradiaphragmatic pituitary adenoma. Bilateral inferior sinus petrosus catheterization and sampling could be of help in the similar cases in order to find the correct localisation of the secreting adenoma.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology