A Rare Case of Adrenocorticotropic Hormone (ACTH)-Secreting Pheochromocytoma Presenting With Severe Cushing Syndrome (CS) and Dual Hormonal Secretion

We present a rare case of a 58-year-old woman diagnosed with an adrenocorticotropic hormone (ACTH)-secreting pheochromocytoma, manifesting as severe Cushing syndrome (CS) with dual-hormonal secretion. She had a three-week history of upper-body tremors, hyperglycemia, hypokalemia, and new-onset, treatment-resistant hypertension. Biochemical investigations revealed markedly elevated cortisol, ACTH, and catecholamine levels. Imaging identified a 3.4 cm enhancing left adrenal mass, and further functional imaging and hormonal assays confirmed the diagnosis. She underwent laparoscopic adrenalectomy following medical stabilization with alpha-blockade and metyrapone. Histopathology confirmed a low-risk pheochromocytoma with positive ACTH immunostaining. Postoperatively, the patient had complete resolution of hypercortisolism, normalization of metabolic parameters, and significant clinical improvement. This case highlights the importance of recognizing dual-hormonal secretion in adrenal tumors, as early detection allows safer preoperative optimization and reduces perioperative risk. It also emphasizes maintaining a high index of suspicion for ectopic ACTH production in rapidly progressing CS. Early diagnosis and appropriate preoperative management are critical to reducing morbidity and mortality.

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Publication Details

Journal
Cureus
Published
2025-11-25
DOI
https://doi.org/10.7759/cureus.97772
Primary Topic
Adrenal and Paraganglionic Tumors
Type
article
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article

A Rare Case of Adrenocorticotropic Hormone (ACTH)-Secreting Pheochromocytoma Presenting With Severe Cushing Syndrome (CS) and Dual Hormonal Secretion

Ye Myint Aung, Robert D. M. Hadden, Tarig Babiker, Sawsan Hamdan et al.
Cureus
Adrenal and Paraganglionic Tumors
article

A Rare Case of Adrenocorticotropic Hormone (ACTH)-Secreting Pheochromocytoma Presenting With Severe Cushing Syndrome (CS) and Dual Hormonal Secretion

Ye Myint Aung, Robert D. M. Hadden, Tarig Babiker, Sawsan Hamdan, N. Hutton
article en

Abstract

We present a rare case of a 58-year-old woman diagnosed with an adrenocorticotropic hormone (ACTH)-secreting pheochromocytoma, manifesting as severe Cushing syndrome (CS) with dual-hormonal secretion. She had a three-week history of upper-body tremors, hyperglycemia, hypokalemia, and new-onset, treatment-resistant hypertension. Biochemical investigations revealed markedly elevated cortisol, ACTH, and catecholamine levels. Imaging identified a 3.4 cm enhancing left adrenal mass, and further functional imaging and hormonal assays confirmed the diagnosis. She underwent laparoscopic adrenalectomy following medical stabilization with alpha-blockade and metyrapone. Histopathology confirmed a low-risk pheochromocytoma with positive ACTH immunostaining. Postoperatively, the patient had complete resolution of hypercortisolism, normalization of metabolic parameters, and significant clinical improvement. This case highlights the importance of recognizing dual-hormonal secretion in adrenal tumors, as early detection allows safer preoperative optimization and reduces perioperative risk. It also emphasizes maintaining a high index of suspicion for ectopic ACTH production in rapidly progressing CS. Early diagnosis and appropriate preoperative management are critical to reducing morbidity and mortality.

CureusVol. 17(11)
Plymouth Hospital (GB), University Hospitals Plymouth NHS Trust (GB)
Openalex Percentile: Top 45%
Adrenal and Paraganglionic Tumors
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