Nonclassic presentation of pheochromocytoma: difficulties in diagnosis and management of the normotensive patient

Abstract

Objective: To report an unusual presentation of pheochromocytoma along with challenges in diagnosis and management.

Methods: We report a clinical case history and describe diagnostic methods and pitfalls. The preoperative medical preparation in a normotensive patient is described.

Results: A 33-year-old man fell off a ladder, resulting in C6 paraplegia. After C7-T1 laminectomy, he was transferred to a rehabilitation center where he reported lightheadedness, shortness of breath, and chest pain during therapy sessions. A left adrenal mass was incidentally discovered during the workup to rule out a pulmonary embolism. He reported no history of hypertension, and no elevated blood pressure readings had been documented. Magnetic resonance imaging of the adrenal glands showed a well-defined left adrenal mass measuring 3.9 x 3.2 x 3.3 cm, which was hyperintense on T2-weighted images. Twenty-four hour urinary catecholamine concentrations were unremarkable; urinary metanephrines were markedly elevated. During hospital admission, blood pressure was in the low to normotensive range, requiring a cautious approach to alpha-adrenergic blockade and surgical preparation. He under-went uneventful laparoscopic left adrenalectomy; surgical pathology was consistent with pheochromocytoma.

Conclusions: This case illustrates a nonclassic presentation of pheochromocytoma and demonstrates that urinary catecholamines alone are not sufficient for a biochemical diagnosis of large pheochromocytomas. Preoperative preparation in normotensive patients can be achieved with alpha-adrenergic blockade, hydration, and liberal salt intake.