Rapidly progressive ACTH-dependent Cushing’s disease masquerading as ectopic ACTH-producing Cushing’s syndrome: illustrative case

Authors

Siyuan Yu, Thomas Jefferson UniversityFollow
Michael Karsy, Thomas Jefferson UniversityFollow
Jeffery Miller, Thomas Jefferson UniversityFollow
Stephanie R. Beldick, Thomas Jefferson UniversityFollow
Mark T. Curtis, Thomas Jefferson UniversityFollow
Marc Rosen, Thomas Jefferson UniversityFollow
James J. Evans, Thomas Jefferson UniversityFollow

Document Type

Article

Publication Date

7-5-2021

Comments

This article is the authors’ final published version in Journal of Neurosurgery: Case Lessons, Volume 2, Issue 1, July 2021, Article number CASE21151.

The published version is available at https://doi.org/10.3171/CASE21151. Copyright © Yu et al.

Publication made possible in part by support from the Jefferson Open Access Fund

Abstract

BACKGROUND Cushing’s disease (CD) remains a challenging condition to diagnose and treat. This case study highlights the challenges of diagnosing CDwhen faced with discrepant clinical, biochemical, and radiologicalfindings.

OBSERVATIONS A 62-year-old man presented with rapid evolution of symptoms, including depression, fatigue, and extreme muscle atrophy, whichresulted in the patient being a wheelchair user over the course of a few months. His rapid clinical course in conjunction with hypercortisolemia in thesetting of a pituitary macroadenoma involving the cavernous sinus, two large pulmonary nodules, and urine-free cortisol levels in the thousandssuggested an aggressive ectopic adrenocorticotropic hormone (ACTH) source. After extensive testing ruled out CD from an ectopic source andbecause of the patient’s abrupt clinical deterioration, the authors concluded that the source was likely an aggressive pituitary adenoma. Therefore, theauthors performed an endonasal transsphenoidal approach for resection of the pituitary adenoma involving the cavernous sinus, and the patient wasscheduled for radiosurgery to control tumor progression.

LESSONS Although extremely high levels of cortisol and ACTH are associated with ectopic Cushing’s syndrome, they may also indicate anaggressive form of CD. Suspicion should be maintained for hypercortisolemia from a pituitary source even when faced with discrepant information thatmay suggest an ectopic source.

Recommended Citation

Yu, Siyuan; Karsy, Michael; Miller, Jeffery; Beldick, Stephanie R.; Curtis, Mark T.; Rosen, Marc; and Evans, James J., "Rapidly progressive ACTH-dependent Cushing’s disease masquerading as ectopic ACTH-producing Cushing’s syndrome: illustrative case" (2021). Department of Neurosurgery Faculty Papers. Paper 158.
https://jdc.jefferson.edu/neurosurgeryfp/158

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This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.

Language

English