Nasser Javadpour (Editor) Principles and Management of Adrenal Cancer With 121 Figures Springer-Verlag London Berlin Heidelberg New York Paris Tokyo Nasser Javadpour. MD. Professor and Director. Section of Urologic Oncology. Department of Surgery. School of Medicine. University of Maryland. University Hospital. Baltimore. Maryland 21201. USA ISBN -l3:978-1-4471-3l36-6 e-ISBN-l3:978-1-4471-3134-2 DOl: 10.1007/978-1-4471-3134-2 British Library Cataloguing in Publication Data Principles and management of adrenal cancer. 1. Adrenal glands-Tumors I. Javadpour. Nasser 616.99'445 RC280.A3 ISBN -13:978-1-4471-313 6-6 Library of Congress Cataloging-in-Publication Data Principles and management of adrenal cancer. Includes bibliographies and index. 1. Adrenal gland----Cancer. I. Javadpour. Nasser. 1937-. [DNLM: 1. Adrenal Gland Neoplasms. WK 780 P957j RC280.A3P75 1987616.99'44586-31443 ISBN -13:978-1-4471-3136-6 This work is subject to copyright. All rights are reserved. whether the whole or part of the material is concerned. specifically the rights of translation. reprinting. re-use of illustrations. recitation. broadcasting. reproduction on microfilms or in other ways. and storage in data banks. Duplication of this publication or parts thereof is only permitted under the provisions of the German Copyright Law of September 9. 1965. in its version of June 24. 1985, and a copyright fee must always be paid. Violations fall under the prosecution act of the German Copyright Law. © Springer-VerIag Berlin Heidelberg 1987 Softcover reprint of the hardcover 1st edition 1987 The use of registered names. trademark etc. in this publication does not imply, even in the absence of a specific statement. that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product Liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature. 2128/3916/543210 Preface The vast amount of literature and rapid developments in the understanding and man agement of adrenal diseases have outpaced the ability of physicians to assimilate and utilize these advances in clinical settings. This book is designed to bring these developments to those interested in adrenal diseases and to assist clinicians in caring for patients with such diseases. The recent advances in the understanding of steroids, catecholamines, and utilization of computed tomography and magnetic resonance imaging have rendered disease of the adrenal gland more rewarding in terms of early detection. Although a number of basic and clinical improvements have been achieved in these diseases, there are still a number of unresolved problems, including the lack of effective cytotoxic agents for therapy of various disseminated adrenal malignancies. The natural history of certain diseases of the adrenal glands and their proximity to the genitourinary system makes these essential organs very attractive to urologic surgeons. Furthermore, for a number of diseases such as adrenogenital syndrome, hypertension, and certain tumors of these glands it is obviously desirable that urologic surgeons are familiar with diseases of "the adrenal glands." The first part of this book is an overview of the relevant embryology, anatomy, physiology, markers, pathology, imaging, and current progress. The second part covers specific diseases of the adrenal cortex and medulla. We hope that this volume will assist the physician in the diagnosis and management of patients with adrenal disease. Baltimore, D.S.A. Nasser ]avadpour February 1987 Contents Chapter 1 Overview of Progress, Current Problems, and Perspectives N. Javadpour. . . . . . . . . . . . . . . 1 Chapter 2 Embryology, Anatomy, Physiology, and Biologic Markers N. Javadpour. . . . . . .. ..... . 15 Chapter 3 Pathology E. E. Lack and H. P. W. Kozakewich . . . . . . . . . . . . 19 Chapter 4 Advances in Diagnosis T.H.Hsu .... 57 Chapter 5 Imaging Techniques D. R. Bodner, C. L. Schultz. and M. I. Resnick ....... 69 Chapter 6 Adrenal Disorders in Childhood S. A. Chalew . . . . . . 83 Chapter 7 Primary Aldosteronism J. H. Mersey . . . . . . . . . . . . . . 95 Chapter 8 Cushing's Syndrome P. A. Levin. . . . . . . . . . . . . . . . . . 107 Chapter 9 Carcinoma A. Zabbo. R. A. Straffon, and J. E. Montie . . . . . . . . . . 113 Chapter 10 Pheochromocytoma B. P. M. Hamilton . 121 Chapter 11 Neuroblastoma B. L6pez-Ibor and A. D. Schwartz . . . . 141 Chapter 12 Metastatic Disease J. H. Mersey . . . . . . . . . . . . . . . . . 153 viii Contents Chapter 13 Surgical Management N. Javadpour . 165 Subject Index. 175 Contributors D. R. Bodner. MD Assistant Professor. Division of Urology. Case Western Reserve University. Cleveland. OH 4416. U.S.A. S. A. Chalew. MD Assistant Professor of Pediatrics. Division of Pediatric Endocrinology. Bressler Building. Rm 10-047. University of Maryland School of Medicine. Baltimore. MD 21201. U.S.A. B. P. M. Hamilton. MD Chief of Endocrinology and Metabolism. Veterans Administration Medical Center. Baltimore. MD 21218. U.S.A. T.H. Hsu. MD Assistant Professor. Department of Medicine. Johns Hopkins Hospital. 600 North Wolfe Street. Baltimore. MD 21205. U.S.A. N. Javadpour. MD Professor and Director. Section of Urologic Oncology. University of Maryland. University Hospital. Baltimore. MD 21201. U.S.A. H. P. W. Kozakewich. MD Assistant Professor of Pathology. Harvard Medical School. Children's Hospital. Boston. MA 02155. U.S.A. E. E. Lack. MD Chief of Surgical Pathology and Post-Mortem Section. National Cancer Institute. National Institutes Of Health. Bethesda. MD 20205. U.S.A. P. A. Levin. MD Assistant Professor of Medicine. Department of Pediatrics. University of Maryland School of Medicine. Baltimore. MD 21201. U.S.A. x Contributors B. L6pez-Ibor. MD Fulbright Fellow in Hematology and Oncology. University of Maryland School of Medicine. Baltimore. MD 21201. U.S.A. J. H. Mersey. MD Assistant Professor. Division of Endocrinology. University of Maryland School of Medicine. Baltimore. MD 21201. U.S-A. J. E. Montie. MD Professor and Chairman. The Department of Urology. The Cleveland Clinic Foundation. 9500 Euclid Avenue. Cleveland. OH 44106. U.S.A. M. I. Resnick. MD Professor and Chairman. Division of Urology. Case Western Reserve University. Cleveland. OH 44106. U.S.A. C. L. Schultz. MD Assistant Professor. Division of Radiology. Case Western Reserve University. Cleveland. OH 4416. U.S.A. A. D. Schwartz. MD Chief of Pediatric Hematology /Oncology. University of Maryland School of Medicine. Baltimore. MD 21201. U.S.A. Chapter 1 Overview of Progress, Current Problems, and Future Perspectives N. Javadpour Recent advances in imaging and localization of a superior renal vein may occasionally be confused adrenal tumors have had a remarkable impact on with the adrenal vein. Even when the adrenal vein their surgical and medical management. Advances is accurately catheterized, the concentration of have also been made in the basic understanding aldosterone will depend on whether the sample is of pituitary and adrenal function, and meaningful obtained proximal or distal to the entrance of the progress has been made in localization of tumors inferior phrenic vein (Fig. 1.1). including venous sampling [8], computed tom ography [9], magnetic resonance imaging, and brush biopsies of the intracaval tumor thrombi. A number of problems remain, the most immediate being a need for effective chemotherapeutic regi mens in the management of local recurrences and disseminated adrenal tumors. In this chapter, I will review briefly the recent advances in the diagnosis and therapy of adrenal diseases with emphasis on surgical problems. Adrenal Vein Sampling The most definitive method of localizing adrenal aldosterone-producing adenomas has been selective adrenal vein catheterization with sampling of aldo sterone concentrations [9]. Adrenal vein sampling is, in most cases, a safe and effective method of localiZing an aldosterone-producing tumor. The technical adequacy of catheter placement in the adrenal vein, particularly on the right, is a critical Fig. 1.1. Catheterization of the left renal vein with injection of factor in the accuracy of this test. On the left side, contrast material. 2 Overview of Progress. Current Problems. and Future Perspectives Fig. 1.3. Catheterization of the left adrenal vein with sampling Fig. 1.2. A small aldosteronoma of the right adrenal gland with and injection of the vein. Note a large hypervascular aldo a moderately hypervascular adenoma. steronoma of the right adrenal gland. On the right side, the main problem is differ entiation of the adrenal vein from the hepatic veins. Small hepatic veins may enter the inferior vena cava at the same level as the adrenal vein. Occasion ally, the right adrenal vein drains into a hepatic or inferior phrenic vein (Figs. 1.2-1.4). The use of the ratio of aldosterone to cortisol concentration in the adrenal vein to that in the inferior vena cava has been helpful in lateralizing adrenal adenoma [18]. By determining the level of cortisol in venous samples, a measure of the dilution of the adrenal efflux may also be gained. The aldo sterone-cortisol ratio then reflects the aldosterone secretion corrected for the degree of selectivity of catheterization. Adrenal cortisol secretion is also unaffected by the aldosterone levels and provides a measure of the degree of accuracy of the adrenal vein sample. Thus, the ratio of aldosterone to cor tisol affords a measure of the hypersecretion of aldo sterone independent of the purity of the sample. Tumor localization is also an integral part of the surgical management of pheochromocytoma. Although the diagnosis may be suspected from the Fig. 1.4. A hypervascular tumor of the left adrenal gland.