Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2018 May 24;2(7):621-630.
doi: 10.1210/js.2018-00086. eCollection 2018 Jul 1.

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Jenan N Gabi  1 , Maali M Milhem  1 , Yara E Tovar  1 , Emhemmid S Karem  2 , Alaa Y Gabi  3 , Rodhan A Khthir  1
Affiliations
Review

Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature

Jenan N Gabi et al. J Endocr Soc. .

Abstract

Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome is rarely caused by a pheochromocytoma. We present a case of a 46-year-old woman who developed severe hypertension, hypokalemia, and typical Cushingoid features. Investigations revealed extremely high metanephrine, cortisol, and ACTH levels. Imaging showed a 3.8-cm left adrenal mass. Preoperative control of hypertension and hypokalemia was very challenging. The patient was cured after surgical removal of the adrenal mass. We followed this by a review of the literature using the databases Google Scholar and PubMed. A total of 58 cases have been reported to date. In summary, ACTH-producing pheochromocytoma is a rare condition that poses a clinical challenge in the perioperative period. It is important that physicians be aware of such a condition because early recognition and treatment are crucial to decrease morbidity and mortality.

Keywords: ACTH-producing pheochromocytoma; Cushing syndrome; ectopic; pheochromocytoma.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Computed tomography scan of the abdomen showing a 3.8-cm left adrenal mass that is partially cystic with enhancing septations.
Figure 2.
Figure 2.
(A) Gross specimen, left adrenal gland. (B) Patchy light brown cytoplasmic staining for ACTH (×200). (C) Nuclear and cytoplasmic staining for chromogranin A (×200).
See this image and copyright information in PMC

References

    1. Newell-Price J, Bertagna X, Grossman AB, Nieman LK. Cushing’s syndrome. Lancet. 2006;367(9522):1605–1617. - PubMed
    1. Sharma ST, Nieman LK, Feelders RA. Cushing’s syndrome: epidemiology and developments in disease management. Clin Epidemiol. 2015;7:281–293. - PMC - PubMed
    1. Lindholm J, Juul S, Jørgensen JO, Astrup J, Bjerre P, Feldt-Rasmussen U, Hagen C, Jørgensen J, Kosteljanetz M, Kristensen L, Laurberg P, Schmidt K, Weeke J. Incidence and late prognosis of cushing’s syndrome: a population-based study. J Clin Endocrinol Metab. 2001;86(1):117–123. - PubMed
    1. Etxabe J, Vazquez JA. Morbidity and mortality in Cushing’s disease: an epidemiological approach. Clin Endocrinol (Oxf). 1994;40(4):479–484. - PubMed
    1. Ballav C, Naziat A, Mihai R, Karavitaki N, Ansorge O, Grossman AB. Mini-review: pheochromocytomas causing the ectopic ACTH syndrome. Endocrine. 2012;42(1):69–73. - PubMed

LinkOut - more resources