Presentation of an unsuspected pheochromocytoma as acute aortic valvular insufficiency and diabetes mellitus type 2

We report the uncommon presentation of an unsuspected pheochromocytoma in a 76-year-old woman. Two years after surgery for acute aortic valve insufficiency and a diagnosis of diabetes, the patient presented with postural headaches, orthostatic dizziness, and palpitations. Physical examination confirmed intractable hypertension, orthostatic hypotension, and congestive heart failure secondary to progressive dilated cardiomyopathy. Based on her symptomatology, examination findings, and progressive dilated cardiomyopathy despite aortic valve replacement, a diagnosis of a pheochromocytoma was entertained. Twenty-four-hour urinary studies confirmed the presence of a catecholamine producing tumor. A magnetic resonance image revealed the presence of a 4.6-cm left adrenal mass, which characteristically enhanced on T2 imaging. After medical stabilization with phenoxybenzamine, she underwent a left robotic adrenalectomy. Histology confirmed a diagnosis of a pheochromocytoma. Two weeks later, she was normoglycemic and normotensive off all medications. The pheochromocytoma is a rare catecholamine-producing tumor, which can be lethal unless recognized and appropriately treated. A high degree of clinical suspicion is necessary, especially in the presence of confounding disease processes. A pheochromocytoma should be considered in the differential diagnosis of acute aortic valvular insufficiency in the setting of orthostatic hypotension, intractable hypertension, and newly diagnosed diabetes.