Adrenocortical carcinoma masquerading as Cushing's disease

Cushing's syndrome (CS) can be classified as adrenocorticotropic hormone (ACTH)-dependent or ACTH-independent depending on the ACTH levels. However, 30% of the patients with CS have ACTH levels in the 'grey zone' (5-20 pg/mL), thereby posing a challenge in establishing the aetiological diagnosis. In a patient with full-blown features of Cushing's syndrome with equivocal ACTH levels, and a pituitary microadenoma on contrast-enhanced MRI sella, can falsely lead to a diagnosis of Cushing's disease. Pituitary microadenoma, if <6 mm in size, may be an incidental finding (incidentaloma) in this scenario and can be present in ∼3-27% of the healthy population. Therefore, in a patient with CS with equivocal ACTH levels and a pituitary microadenoma, multiple samplings for ACTH and adrenal imaging should be performed to exclude ACTH-independent CS and if required, bilateral inferior petrosal sinus sampling to determine the source of ACTH excess.