Ectopic ACTH-secreting pituitary adenomas within the sphenoid sinus

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...

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Bibliographic Details
Published in:Endocrine 2014-12, Vol.47 (3), p.717-724
Main Authors: Johnston, Philip C., Kennedy, Laurence, Weil, Robert J., Hamrahian, Amir H.
Format: Article
Language:English
Subjects:
ACTH Syndrome, Ectopic - diagnosis
ACTH Syndrome, Ectopic - pathology
ACTH-Secreting Pituitary Adenoma - pathology
Adenoma - pathology
Diabetes
Endocrinology
Humanities and Social Sciences
Humans
Internal Medicine
Medicine
Medicine & Public Health
Mini Review
multidisciplinary
Paranasal Sinus Neoplasms - pathology
Science
Sphenoid Sinus - pathology
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Summary: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.
ISSN:1355-008X
1559-0100
DOI:10.1007/s12020-014-0313-z