An unusual case of Adrenocortical Adenocarcinoma with Biochemical Masquerade of Pheochromocytoma

Adrenocortical carcinoma (ACC) is a rare malignancy that arises from the adrenal cortex and often presents as adrenal incidentaloma on abdominal scans with rise in the use of imaging modalities. ACC often presents as Cushing’s syndrome or virilization. On the other hand, pheochromocytoma is an adrenal medullary tumor. It is rare for ACC to present as pheochromocytoma even though both may coexist. Moreover, ACC tumors have radiological and histological features suggestive of aggressive nature of the disease. We present a case of a 65-year-old lady who initially presented with a 3cm left adrenal incidentaloma. All of her adrenal hormones were in normal range. She was lost to follow up for several years and returned with a much enlarged lesion. Biochemical work up showed mildly increased catecholamines and metanephrines suggestive of pheochromocytoma. She didn’t have any signs or symptoms of pheochromocytoma. She was treated with alpha blockers before surgery as a prophylactic measure. Surgical pathology was consistent with the diagnosis of primary adrenal adenocarcinoma. We recommend that adrenal incidentalomas should be followed annually for up to five years as per American association of Endocrinology and the Endocrine Society guidelines to prevent morbidity and mortality in patients.