A Hormonal Storm Behind the Heart: ACTH-Producing Pheochromocytoma Presenting with Acute Coronary Syndrome and Diabetic Ketoacidosis

Abstract

Background

Clinical manifestations of pheochromocytoma can be highly variable and life-threatening, particularly affecting the endocrine and cardiovascular systems. We report an unusual case of adrenal pheochromocytoma.

Case Description

A 54-year-old woman with long-standing diabetes and prior cardiovascular events presented with episodic chest pain and labile blood glucose levels. Imaging revealed a left adrenal mass and biochemical studies confirmed excessive catecholamine and adrenocorticotropic hormone (ACTH) secretion, with failure of cortisol suppression. Adrenalectomy confirmed pheochromocytoma with ectopic ACTH production. Postoperatively, the patient achieved complete biochemical remission and glycemic stability on metformin monotherapy.

Conclusion

This case highlights the diagnostic complexity of pheochromocytoma with atypical presentations. ACTH cosecretion can mimic Cushing's syndrome and precipitate metabolic crises such as diabetic ketoacidosis. Early recognition and surgical intervention are essential for improving clinical outcomes and achieving long-term stability.

Keywords

pheochromocytoma - ACTH secretion - diabetic ketoacidosis - acute coronary syndrome - adrenal tumor - endocrine disorders

Patient's Consent Statement

The patient gave informed consent for the publication of this case report and accompanying images. The report was anonymized to protect the patient's identity.

Authors' Contributions

A.A.R., I.A., S.A.G.: Conceptualization, data collection, clinical management, manuscript writing, and final approval.

Ethical Considerations

No prior ethical approval is required for single case reports and small series.

Publikationsverlauf

Artikel online veröffentlicht:
29. Juli 2025

• References

• 1 Young Jr WF. Clinical practice. the incidentally discovered adrenal mass. N Engl J Med 2007; 356 (06) 601-610
• 2 Meijs AC, Snel M, Corssmit EPM. Pheochromocytoma/paraganglioma crisis: case series from a tertiary referral center for pheochromocytomas and paragangliomas. Hormones (Athens) 2021; 20 (02) 395-403
• 3 Patel D. Surgical approach to patients with pheochromocytoma. Gland Surg 2020; 9 (01) 32-42
• 4 Prejbisz A, Lenders JWM, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. J Hypertens 2011; 29 (11) 2049-2060
• 5 Gu YW, Poste J, Kunal M, Schwarcz M, Weiss I. Cardiovascular manifestations of pheochromocytoma. Cardiol Rev 2017; 25 (05) 215-222
• 6 Gabi JN, Milhem MM, Tovar YE, Karem ES, Gabi AY, Kathir RA. Severe Cushing syndrome due to an ACTH-producing pheochromocytoma: a case and literature review. J Endocr Soc 2018; 2 (07) 621-630
• 7 Giraud R, Glauser A, Looyens C. et al. Pheochromocytoma multisystem crisis requiring mechanical circulatory support: a narrative review. J Clin Med 2025; 14 (06) 1907
• 8 Bobrowicz M, Nagórska A, Karpiłowska A. et al. Pheochromocytoma-induced pseudo-Cushing's syndrome: case report. Front Endocrinol (Lausanne) 2024; 15: 1491873
• 9 Elliott PF, Berhane T, Ragnarsson O, Falhammar H. Ectopic ACTH- and/or CRH-producing pheochromocytomas. J Clin Endocrinol Metab 2021; 106 (02) 598-608
• 10 Terzolo M, Alì A, Pia A. et al. Cyclic Cushing's syndrome due to ectopic ACTH secretion by an adrenal pheochromocytoma. J Endocrinol Invest 1994; 17 (11) 869-874
• 11 Ishii C, lnoue K, Negishi K, Tane N, Awata T, Katayama S. Diabetic ketoacidosis in a case of pheochromocytoma. Diabetes Res Clin Pract 2001; 54 (02) 137-142
• 12 Lenders JWM, Duh QY, Eisenhofer G. et al; Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014; 99 (06) 1915-1942
• 13 White A, Ray DW, Talbot A, Abraham P, Thody AJ, Bevan JS. Cushing's syndrome is due to phaeochromocytoma secreting the precursors of ACTH. J Clin Endocrinol Metab 2000; 85 (12) 4771-4775