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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.

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Acknowledgments

We would like to thank Dr. Richard Prayson and Dr. Daniel Lockwood for providing the histological pictures and pituitary imaging, respectively.

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Correspondence to Amir H. Hamrahian.

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Johnston, P.C., Kennedy, L., Weil, R.J. et al. Ectopic ACTH-secreting pituitary adenomas within the sphenoid sinus. Endocrine 47, 717–724 (2014). https://doi.org/10.1007/s12020-014-0313-z

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Keywords

  • Ectopic corticotroph adenoma
  • Ectopic pituitary adenoma
  • Cushing’s syndrome
  • Sphenoid sinus
  • Anatomy
  • Transsphenoidal surgery