Adrenocorticotropin-Secreting Pure Adrenal Ganglioneuroma Leading to Cushing Syndrome

Adrenal ganglioneuromas (AGNs) are rare benign neoplasms of neural crest origin that are typically asymptomatic and endocrinologically inactive. However, on occasion, AGNs have been noted to demonstrate secretion of various hormones. We present a case of a 23-year-old man who presented with hypertension and clinical symptoms concerning for a catecholamine excess state with evidence of a right adrenal mass with elevated Hounsfield units (HU) on abdominal computed tomography (CT) and laboratory values consistent with mild hypercortisolism concerning for adrenocorticotropin (ACTH)-producing pheochromocytoma leading to Cushing syndrome (CS). The patient underwent adrenalectomy with pathology results demonstrating a pure AGN with positive ACTH staining. While secretory AGNs are rare, it is important that providers remain cognizant of this pathology and consider it within the evaluation of adrenal lesions at large with an understanding that clinical presentation may be difficult to interpret and will likely vary based on the underlying hormone(s) being secreted. Additionally, secretory AGNs can mimic other adrenal neoplasms and histopathologic evaluation is required for definitive diagnosis. This case demonstrates how ACTH-producing AGNs should be considered when evaluating cases of ACTH-dependent CS.