title: Ectopic ACTH-secreting pituitary adenomas within the sphenoid sinus.
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 ACTH-dependent 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.
authors.affiliation: Department of Endocrinology, Diabetes and Metabolism, Cleveland Clinic Foundation, 9500 Euclid Avenue Desk F20, Cleveland, OH, 44195, USA.
authors.forename: Philip C
authors.initials: PC
authors.lastname: Johnston
authors.forename: Laurence
authors.initials: L
authors.lastname: Kennedy
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Weil
authors.forename: Amir H
authors.initials: AH
authors.lastname: Hamrahian
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000175:diagnosis* / Q000473:pathology; D049913:ACTH-Secreting Pituitary Adenoma / Q000473:pathology*; D000236:Adenoma / Q000473:pathology*; D006801:Humans; D010255:Paranasal Sinus Neoplasms / Q000473:pathology*; D013101:Sphenoid Sinus / Q000473:pathology*