Ectopic ACTH-secreting pituitary adenomas within the sphenoid sinus
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MINI REVIEW
Ectopic ACTH-secreting pituitary adenomas within the sphenoid sinus Philip C. Johnston • Laurence Kennedy Robert J. Weil • Amir H. Hamrahian
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Received: 11 February 2014 / Accepted: 21 May 2014 Ó Springer Science+Business Media New York 2014
Abstract Ectopic pituitary adenomas are defined by the presence of adenomatous pituitary tissue outside the sella and distinctly separate from the pituitary gland. Ectopic ACTH-secreting pituitary adenomas (EAPAs) are a rare cause of Cushing’s syndrome. Detecting these radiologically can prove difficult, in part, due to their typically small size and unpredictable anatomical location. In ACTHdependent Cushing’s syndrome, if, despite comprehensive testing, the source of excess ACTH remains occult (including negative work up for ectopic ACTH syndrome) thought should be given to the possibility of the patient harboring an EAPA. In most cases, ectopic ACTH pituitary adenomas within the sphenoid sinus will manifest with symptoms of hormonal excess, have an obvious sphenoid sinus mass on pre-operative imaging and will demonstrate resolution of hypercortisolism after surgical excision if located and removed. Twenty cases of EAPAs have been reported in the literature to date. This paper will review the current literature on all previously reported EAPAs within the sphenoid sinus in addition to the current case.
P. C. Johnston L. Kennedy A. H. Hamrahian (&) Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, OH 44195, USA e-mail: [email protected] R. J. Weil Department of Neurosurgery and the Neurological Institute, Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk ND40, Cleveland, OH 44195, USA Present Address: R. J. Weil Department of Neurosurgery, Geisinger Health System, 100 N Academy Dr, Danville, PA 17801, USA
Keywords Ectopic corticotroph adenoma Ectopic pituitary adenoma Cushing’s syndrome Sphenoid sinus Anatomy Transsphenoidal surgery
Introduction Cushing’s disease is rare and results from excess cortisol due to hypersecretion of ACTH by corticotroph adenomas, with an incidence of *0.7 to 2.4 cases per million persons/ year. It is associated with increased morbidity and mortality if untreated [1, 2]. Potential sequelae include diabetes mellitus, obesity, hypertension, osteoporosis, depression, opportunistic infections, and increased cardiovascular mortality [3, 4]. In ACTH-dependent Cushing’s syndrome, ACTH can originate from either a pituitary adenoma or ectopic ACTH syndrome, which most commonly includes neuroendocrine tumors of the lung, carcinoid, thyroid, pancreas, prostate, and small cell carcinoma of the lung [5– 7]. A less common etiology is an ectopic ACTH-secreting pituitary adenoma [8–10]. Possible explanations for ectopic pituitary tissue in and around the sella include embryological mechanisms–either from residual ectodermal cell rests deposited along the path of formation of the pituitary gland or aberr
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