Cushing Syndrome Due to a Corticotropin-Releasing Hormone– and Adrenocorticotropic Hormone–Secreting Silent Pheochromocytoma

Sananda Moctezuma, MD; Jonathan L. Perez, BS; Ezra Baraban, MD; Patrizio Caturegli, MD, MPH; Lilah Morris-Wiseman, MD; Roberto Salvatori, MD

AACE Clinical Case Reports (May 2024)

Cushing Syndrome Due to a Corticotropin-Releasing Hormone– and Adrenocorticotropic Hormone–Secreting Silent Pheochromocytoma

Sananda Moctezuma, MD,
Jonathan L. Perez, BS,
Ezra Baraban, MD,
Patrizio Caturegli, MD, MPH,
Lilah Morris-Wiseman, MD,
Roberto Salvatori, MD

Affiliations

Sananda Moctezuma, MD: Division of Endocrinology, Department of Medicine, Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, Maryland
Jonathan L. Perez, BS: Department of Pathology, Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, Maryland
Ezra Baraban, MD: Department of Pathology, Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, Maryland
Patrizio Caturegli, MD, MPH: Department of Pathology, Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, Maryland
Lilah Morris-Wiseman, MD: Department of Surgery, Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, Maryland
Roberto Salvatori, MD: Division of Endocrinology, Department of Medicine, Johns Hopkins University School of Medicine, Johns Hopkins University, Baltimore, Maryland; Address correspondence to Dr Roberto Salvatori, Division of Endocrinology, Diabetes and Metabolism, 1830 East Monument Street #333, Baltimore, MD 21287.

Journal volume & issue: Vol. 10, no. 3
pp. 84 – 88

Abstract

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Background/Objective: Ectopic cosecretion of corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH) in silent (ie, non–catecholamine-secreting) pheochromocytoma is a rare cause of Cushing syndrome. Case Report: A 57-year-old woman rapidly developed hypercortisolism, clinically manifesting as fatigue, muscle weakness, weight gain, and worsening hypertension and biochemically characterized by hypokalemia and marked increases in the serum cortisol and plasma ACTH levels. This acute presentation suggested a diagnosis of ectopic ACTH syndrome (EAS). Imaging studies revealed a right adrenal mass that enhanced after administration of the radioisotope gallium-68-DOTATATE. Plasma metanephrines were normal in 2 separate measurements. The possibility of a silent pheochromocytoma was considered. After controlling her hypercortisolism with metyrapone and surgical preparation with alpha blockade, the patient underwent elective right adrenalectomy. Pathology revealed a pheochromocytoma that stained focally for ACTH and CRH. Postoperatively, the cortisol levels normalized, the hypothalamic-pituitary-adrenal axis was not suppressed, and clinical symptoms from hypercortisolism abated. Discussion: Patients who exhibit a rapid progression of ACTH-dependent hypercortisolism should be screened for EAS. The use of functional imaging radioisotopes (eg, gallium DOTA-peptides) improves the detection of ACTH-secreting tumors. Preoperative treatment with steroidogenesis inhibitors helps control clinical and metabolic derangements associated with severe hypercortisolemia, whereas alpha blockade prevents the onset of an adrenergic crisis. Conclusion: We present a rare case of EAS due to a silent pheochromocytoma that cosecreted ACTH and CRH. Pheochromocytoma should be considered in patients with EAS who have an adrenal mass even in the absence of excessive catecholamine secretion.

Published in AACE Clinical Case Reports

ISSN: 2376-0605 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the endocrine glands. Clinical endocrinology
Website: https://www.journals.elsevier.com/aace-clinical-case-reports

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