• Ulrich J Knappe, Christian Jaspers, Desirée Buschsieweke, Wolf-Dieter Reinbold, Ali Alomari, Wolfgang Saeger, Klaus Ehlenz, W Alexander Mann, Peter Herbert Kann, and Joachim Feldkamp.
• Department of Neurosurgery, Johannes Wesling Klinikum, Minden, Germany.
• Neurosurgery. 2017 Apr 1; 80 (4): 525-533.

BackgroundThe diagnosis of Cushing disease is based on endocrinological pa-rameters, with no single test being specific. In some patients, dynamic thin-slice sellar magnetic resonance imaging fails to detect a pituitary tumor.ObjectiveThe purpose of this study is to investigate the role of ectopic pituitary adenoma in this situation.MethodsIn a retrospective chart review, 5 patients (6%) with ectopic adenomas were identified in 83 consecutive patients undergoing transsphenoidal surgery for adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas by 1 surgeon.ResultsIn all 5 patients (all female, 32-41 years of age), an exclusively extrasellar ACTH-secreting adenoma was excised. Three adenomas were located in the cavernous sinus, 1 in the sphenoid sinus, and 1 in the ethmoidal cells. Histologically, none of the tumors showed signs of aggressiveness. Three of the 5 adenomas specifically expressed somatostatin receptor 5. In 4 patients with Cushing disease, postoperative remission was obtained, with 1 recurrence after 14 months. In the patient with Nelson syndrome, ACTH decreased from >800 to <80 pg/mL. Three patients underwent previous surgery elsewhere, including 1 hypophysectomy. In this case, the ectopic adenoma (positive for somatostatin receptor 5) in the ethmoidal cells turned out to be positive on gallium 68 DOTATATE positron emission tomography/computed tomography.ConclusionThe incidence of primarily ectopic ACTH-secreting adenomas in this series was 6%. In cases of negative MRI findings, an ectopic ACTH-secreting adenoma should be taken into account. 68 Ga DOTATATE positron emission tomography/computed tomography may identify ectopic pituitary adenomas. Hypophysectomy should always be avoided in primary surgery for CD.Copyright © 2016 by the Congress of Neurological Surgeons

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