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Silent Adrenal Nodules in von Hippel-Lindau Disease Suggest Pheochromocytoma

Brian S. Aprill, MD; Almond J. Drake, MD; David H. Lasseter, MD; and K. M. Mohamed Shakir, MD
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From the National Naval Medical Center and the Uniformed Services University of the Health Sciences, Bethesda, Maryland. Requests for Reprints: Captain K.M.M. Shakir, MC, USN, Division of Endocrinology and Metabolism, Department of Internal Medicine, National Naval Medical Center, Bethesda, Maryland 20889-5600. Acknowledgment: The authors thank Pat Rattal for editorial assistance.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1994;120(6):485-487. doi:10.7326/0003-4819-120-6-199403150-00006
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Von Hippel-Lindau disease is an autosomal-dominant disorder characterized by retinal angiomatosis, cerebellar hemangioblastoma, renal cell carcinoma, and, less commonly, pheochromocytoma. Lindau described adrenal tumors in association with this syndrome in 1927 [1]. Not until 1953, however, did Glushien and coworkers [2] first report pheochromocytoma in association with von Hippel-Lindau disease. A 1987 review summarized 338 cases of this disease in 62 kindreds in which pheochromocytoma was reported in 14% [3]. Patients with von Hippel-Lindau disease who have a renal mass and clinically silent adrenal nodule often are difficult to treat, especially if renal surgery is needed. Physicians should strongly consider the diagnosis of pheochromocytoma in these patients, because of the increased operative risk, and plan for concurrent renal and ipsilateral adrenal resections to avoid another operation when pheochromocytoma becomes clinically evident. We describe four patients with von Hippel-Lindau disease in whom clinically silent adrenal nodules were found; in three of them, detailed evaluation and observation showed pheochromocytoma.

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