Association of Pheochromocytoma and Ganglioneuroma: Unusual Finding in Neurofibromatosis Type 1 |
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| Institution: | 1. From the Department of Medicine, Division of Endocrinology, New York-, Presbyterian Hospital/Weill-Cornell Medical Center and Lenox Hill Hospital, New York, New York;2. From the Department of Microbiology-Immunology, Medical School of Teresopolis, Rio de Janeiro, Brazil;3. From the Section of Clinical Nutrition, Memorial Sloan Kettering Cancer Center, New York, New York,;4. From the Department of Urology, New York-Presbyterian Hospital/Weill-Cornell Medical Center, New York, New York, and;5. From the Department of Pathology and Laboratory Medicine, New York-Presbyterian Hospital/Weill-Cornell Medical Center, New York, New York.;1. Department of Dermatology, Temple University Lewis Katz School of Medicine, Philadelphia, Pennsylvania, USA;2. Department of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania, USA;3. Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida, USA |
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| Abstract: | ObjectiveTo report a rare case of association of pheochromocytoma and ganglioneuroma in an asymptomatic patient with neurofibromatosis type 1 (NF1) and to discuss the importance of annual biochemical and imaging studies.MethodsWe present the clinical, laboratory, and pathology findings in a 41-year-old woman with NF1 and review the pertinent literature.ResultsA 41-year-old woman with NF1 presented for a routine gynecologic examination, at which time a right adrenal mass (4 by 3 cm) was discovered by abdominal ultrasonography and confirmed by abdominal computed tomographic scans and magnetic resonance imaging. The patient was normotensive and complained only of discrete essential tremors. Biochemical studies showed a serum epinephrine level of 195 pg/mL (normal,<100) and a 24-hour urine epinephrine excretion of 55 μg (normal,<20), findings consistent with pheochromocytoma. Metaiodobenzylguanidine scintigraphy revealed uptake in the right adrenal gland, with no evidence of metastatic lesions. Before surgical treatment, the patient received an α-adrenergic antagonist for 30 days. Laparoscopic excision of the right adrenal gland yielded excellent postoperative results. Surgical pathology revealed a multinodular mass composed of pheochromocytoma and ganglioneuroma. In patients with NF1 (von Recklinghausen’s disease), a tumor consisting of pheochromocytoma and ganglioneuroma is rare and may be more aggressive than pheochromocytoma alone. An asymptomatic catecholamine-producing tumor may cause substantial morbidity and mortality, especially in patients who are undergoing surgical intervention or are under other stressors.ConclusionThe current guidelines for managing patients with NF1 are an annual history and physical examination. Because of the increased prevalence of pheochromocytoma and ganglioneuroma in patients with NF1, and the potential associated adverse effects, we emphasize the importance of periodic clinical evaluation with biochemical testing and imaging studies. (Endocr Pract. 2007;13:647-651) |
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