A Difficult Case of Ectopic ACTH Syndrome: Is Treatment Possible Even Without Accurate Localization?

Adam I Kaplan; Liam Clifford; Ammar Wakil

doi:10.15605/jafes.040.02.26

Authors

Adam I Kaplan Gosford Hospital https://orcid.org/0000-0001-5824-2991
Liam Clifford Gosford Hospital, Gosford, New South Wales, Australia
Ammar Wakil Wyong Hospital, Wyong. New South Wales, Australia

DOI:

https://doi.org/10.15605/jafes.040.02.26

Keywords:

ectopic ACTH syndrome, Cushing syndrome, hypercortisolism, ketoconazole

Abstract

Occult ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a relatively rare form of ACTH-dependent Cushing’s Syndrome (CS). We describe a middle-aged female with symptomatic CS, where despite thorough investigations including hormone suppression testing, multiple imaging modalities, and inferior petrosal sinus sampling (IPSS), an ectopic source could not be demonstrated. Our case demonstrates that ketoconazole can be an effective therapeutic to suppress cortisol production even when the source of ectopic ACTH production is unknown. However, long-term evaluation is required.

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Author Biographies

Adam I Kaplan, Gosford Hospital

Department of Endocrinology, Gosford Hospital, Gosford, New South Wales, Australia
School of Medicine and Public Health, The University of Newcastle, New South Wales, Australia

Liam Clifford, Gosford Hospital, Gosford, New South Wales, Australia

Department of Endocrinology, Gosford Hospital, Gosford, New South Wales, Australia

Ammar Wakil, Wyong Hospital, Wyong. New South Wales, Australia

Department of General Medicine, Wyong Hospital, Wyong. New South Wales, Australia

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