Adrenocorticotropic Hormon-Secreting Pheochromocytoma: A Rare Cause of Cushing's Syndrome

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Bolayir B., Okay D., Akturk M. Y. , AKIN M., POYRAZ A., Sel A. T. , ...More

TURKISH JOURNAL OF ENDOCRINOLOGY AND METABOLISM, vol.23, no.2, pp.125-129, 2019 (ESCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 23 Issue: 2
  • Publication Date: 2019
  • Doi Number: 10.25179/tjem.2018-63374
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.125-129
  • Keywords: Ectopic Cushing's syndrome, pheochromocytoma, ACTH staining
  • Gazi University Affiliated: Yes


Pheochromocytoma is a rare cause of ectopic Cushing's syndrome and presents a challenge to the clinician in the diagnosis and treatment. In this study, a rare case of adrenocorticotropic hormone-producing pheochromocytoma is presented. A 55-years-old man with the symptoms of hypercortisolism, multidrug-resistant hypertension, and an adrenal mass is presented. The laboratory results were consistent with hypercortisolism, high plasma adrenocorticotropic hormone, high 24-hour urine metanephrine and normetanephrine and severe hypokalemia. Abdominal computerized tomography showed a 3-cm non-adenoma left adrenal mass. After preoperative management, left adrenalectomy was performed. A histopathological examination revealed a 2.5 cm pheochromocytoma with focal positivity for adrenocorticotropic hormone. The patient was discharged with full recovery under hydrocortisone replacement therapy. Furthermore, six weeks after the operation, 24-hour urinary excretion of metanephrine and normetanephrine was within normal ranges. In conclusion, an adrenocorticotropic hormone-producing pheochromocytoma should be considered in patients with clinical manifestations of ectopic Cushing's syndrome and adrenal mass.