Nonclassic Presentation of Pheochromocytoma: Difficulties in Diagnosis and Management of the Normotensive Patient |
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| Affiliation: | 1. Senior Vice President, Dean of the Medical Senior Vice President, Dean of the Medical Faculty, Helene A. and Philip E. Hixon Chair in Investigative Medicine, Cedars-Sinai Medical Center, Los Angeles, CA.;2. Professor, Director Neuro Endocrine Unit, Department of Medicine, NYU Langone Medical Center, New York, New York.;3. Clinical Director, Pituitary Center, Cedars-Sinai Medical Center, Los Angeles, CA.;4. Associate Professor of Medicine, (Endocrinology) and Neurological Surgery, Director Northwest Pituitary Center, Oregon Health & Science University, Portland, OR.;1. Section of Endocrine Surgery, UCLA David Geffen School of Medicine, Los Angeles, California.;2. Department of Pathology and Laboratory Medicine, UCLA David Geffen School of Medicine, Los Angeles, California.;1. Nuclear Medicine Unit, Department of Medical-Surgical Sciences and of Translational Medicine, “Sapienza” University of Rome, Rome, Italy;2. Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy |
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| Abstract: | ObjectiveTo report an unusual presentation of pheochromocytoma along with challenges in diagnosis and management.MethodsWe report a clinical case history and describe diagnostic methods and pitfalls. The preoperative medical preparation in a normotensive patient is described.ResultsA 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 × 3.2 × 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 α-adrenergic blockade and surgical preparation. He underwent uneventful laparoscopic left adrenalectomy; surgical pathology was consistent with pheochromocytoma.ConclusionsThis 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 α-adrenergic blockade, hydration, and liberal salt intake. |
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