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Review
. 2022 Apr;55(2):315-329.
doi: 10.1016/j.otc.2021.12.006. Epub 2022 Mar 4.

Cushing Disease: Medical and Surgical Considerations

David P Bray  1 , Rima S Rindler  2 , Reem A Dawoud  3 , Andrew B Boucher  4 , Nelson M Oyesiku  5
Affiliations
Review

Cushing Disease: Medical and Surgical Considerations

David P Bray et al. Otolaryngol Clin North Am. 2022 Apr.

Abstract

Cushing disease is a disorder of hypercortisolemia caused by hypersecretion of adrenocorticotropic hormone by a pituitary adenoma and is a rare diagnosis. Cushing disease presents with characteristic clinical signs and symptoms associated with excess cortisol, but diagnosis is difficult and often relies on repeated and varied endocrinologic assays and neuroradiologic investigations. Gold standard treatment is surgical resection of adrenocorticotropic hormone-secreting pituitary adenoma, which is curative. Patients require close endocrinologic follow-up for maintenance of associated neuroendocrine deficiencies and surveillance for potential recurrence. Medications, radiation therapy, and bilateral adrenalectomy are alternative treatments for residual or recurrent disease.

Keywords: Adenoma; Cushing disease; Endonasal; Endoscopic; Pituitary; Skull base; Transnasal.

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Conflict of interest statement

Disclosure of funding Dr. David P. Bray is partly supported by the Nell W. and William S. Elkin Research Fellowship in Oncology, Winship Cancer Institute, Emory University Hospital, Atlanta, GA and supported in part by the National Center for Advancing Translational Sciences of the National Institutes of Health under Award Numbers UL1TR002378 and TL1TR002382.

Figures

Figure 1:
Figure 1:
A flowchart demonstrating the sequence of testing required for diagnosis of Cushing’s Disease.
Figure 2:
Figure 2:
Case of a 40-year-old woman with Cushing’s Disease. A, B, C) MRI of the brain, sella protocol showing a pituitary adenoma in the left anterior aspect of the pituitary gland within the sella. It is T1 non-enhancing (A coronal, B sagittal) with a small T2 hyperintense component (C coronal). D) There was a significant drop in morning cortisol following surgical resection from 28 mcg/dL to 0.9 mcg/dL by postoperative day 2.
Figure 3:
Figure 3:
Case of a 49-year-old man with Cushing’s Disease and a “double adenoma.” A, B) MRI of the brain, sella protocol showing a pituitary adenoma (white arrow) in the anterior right aspect of the pituitary gland abutting the cavernous sinus (A coronal, B sagittal). C) central:peripheral cortisol ratios from the right and left side of the pituitary gland during inferior petrosal sinus sampling, indicating higher relative ACTH levels from the right gland. D) Pre- and postoperative serum cortisol levels for first transsphenoidal resection of right pituitary adenoma, indicating persistently elevated levels. E) Pre- and postoperative serum cortisol levels for second transsphenoidal exploration, discovery and resection of left pituitary adenoma, demonstrating cortisol nadir postoperatively to normal levels.
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References

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