Silent Adrenal Nodules in von Hippel-Lindau Disease Suggest Pheochromocytoma
Authors: Brian S. Aprill
Journal: Annals of Internal Medicine (1994)

Brief Reports15 March 1994Silent Adrenal Nodules in von Hippel-Lindau Disease Suggest PheochromocytomaBrian S. Aprill, MD, Almond J. Drake, MD, David H. Lasseter, MD, and K. M. Mohamed Shakir, MDBrian S. Aprill, MDFrom the National Naval Medical Center and the Uniformed Services University of the Health Sciences, Bethesda, Maryland.Search for more papers by this author, Almond J. Drake, MDFrom the National Naval Medical Center and the Uniformed Services University of the Health Sciences, Bethesda, Maryland.Search for more papers by this author, David H. Lasseter, MDFrom the National Naval Medical Center and the Uniformed Services University of the Health Sciences, Bethesda, Maryland.Search for more papers by this author, and K. M. Mohamed Shakir, MDFrom the National Naval Medical Center and the Uniformed Services University of the Health Sciences, Bethesda, Maryland.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-120-6-199403150-00006 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail 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 ...References1. Melmon KL, Rosen SW. Lindau's Disease—review of the literature and study of a large kindred. Am J Med. 1964; 36:595-617. Google Scholar2. Glushien AS, Mansuy MM, Littman DS. Pheochromocytoma—its relationship to the neurocutaneous syndromes. Am J Med. 1953; 14: 318-27. Google Scholar3. Case records of the Massachusetts General Hospital (case 16-1991). N Engl J Med. 1991; 324:1119-27. Google Scholar4. Shapiro B, Copp JE, Sisson JC, Eyre PL, Wallis J, Beierwaltes WH. Iodine-131 metaiodobenzylguanidine for the locating of suspected pheochromocytoma: experience in 400 cases. J Nucl Med. 1985; 26: 576-85. Google Scholar5. Grossman E, Goldstein DS, Hoffman A, Keiser HR. Glucagon and clonidine testing in the diagnosis of pheochromocytoma. Hypertension. 1991; 17:733-41. Google Scholar6. Siqueira-Filho AG, Sheps SG, Maher FT, Jiang NS, Elveback LR. Glucagon-blood catecholamine test: use in isolated and familial pheochromocytoma. Arch Intern Med. 1975; 135:1227-31. Google Scholar7. Renal mass in a man with von-Hippel Lindau disease (clinical conference). Am J Med. 1981; 71:287-97. Google Scholar8. Elliott WJ, Murphy MB. Reduced specificity of the clonidine suppression test in patients with normal plasma catecholamine levels. Am J Med. 1988; 84:419-24. Google Scholar Author, Article, and Disclosure InformationAffiliations: From the National Naval Medical Center and the Uniformed Services University of the Health Sciences, Bethesda, Maryland.Corresponding Author: 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. 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Goldstein, MD, PhDPheochromocytomaMANAGEMENT APPROACHES TO ADRENAL INCIDENTALOMASCLINICAL AND GENETIC CHARACTERIZATION OF PHEOCHROMOCYTOMA IN VON HIPPEL-LINDAU FAMILIES: COMPARISON WITH SPORADIC PHEOCHROMOCYTOMA GIVES INSIGHT INTO NATURAL HISTORY OF PHEOCHROMOCYTOMAPlasma Normetanephrine and Metanephrine for Detecting Pheochromocytoma in von Hippel–Lindau Disease and Multiple Endocrine Neoplasia Type 2Surgical Decision-Making Affected By Clinical and Genetic Screening of a Novel Kindred With Von Hippel-Lindau Disease and Pancreatic Islet Cell TumorsCortical-sparing adrenalectomy for patients with bilateral pheochromocytomaPheochromocytoma: Update on Diagnosis, Localization, and Management 15 March 1994Volume 120, Issue 6Page: 485-487KeywordsAdrenalectomyBlood plasmaBlood pressureCatecholaminesComputed axial tomographyHypertensionMagnetic resonance imagingPheochromocytomaRenal diseasesUrine Issue Published: 15 March 1994 Copyright & PermissionsCopyright © 1994 by American College of Physicians. 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