Endocrine Abstracts

Introduction: Endogenous hypercortisolism (EH) is a serious condition caused by an excess of glucocorticoids in the body, classified into ACTH-dependent and ACTH-independent variants, occurring in 70-80% and 20-30% of cases, respectively. A rare cause of ACTH-dependent EH is ectopic ACTH syndrome (ACTH-ES), accounting for approximately 15-20% of cases. ACTH-ES is characterized by the hyperproduction of adrenocorticotropic hormone (ACTH) from neuroendocrine tumors outside the pituitary gland, including pheochromocytoma (PC).

Case Report: We present the first case of Cushing’s disease associated with pheochromocytoma in the Colombian Northwest. The patient, a male in his thirties, exhibited clinical signs of hypercortisolism and sought care at the region’s most complex hospital. Laboratory tests revealed urinary cortisol levels exceeding five times the normal range, with normal ACTH levels. Further examination confirmed ectopic Cushing’s syndrome (CS). Multislice computed tomography (MSCT) of the abdomen identified a mass in the left adrenal gland. Urine tests showed a significant increase in methylated catecholamine derivatives. The patient underwent left adrenalectomy, and the diagnosis of pheochromocytoma was confirmed morphologically and pathologically. An immunohistochemical study demonstrated intense expression of chromogranin A and ACTH in the tumor cells.

Discussion: This case highlights the rare occurrence of paraneoplastic ACTH production by pheochromocytoma. The patient’s presentation with hypercortisolism and the confirmation of ectopic ACTH production emphasize the complexity of diagnosing adrenal tumors.

Conclusion: This report underscores the importance of considering pheochromocytoma in patients with hypercortisolism and elevated urinary cortisol levels. Early identification and surgical intervention are crucial for effective management and improved patient outcomes.