PHEOCHROMOCYTOMA William Muir Manger Ray W. Gifford, Jr. PHEOCHROMOCYTOMA with 132 figures and 28 color pia tes Springer-Verlag New York Heidelberg Berlin Wi.lliam Muir Manger, M.D., Ph.D., FAC.P., FAC.C. Associate Professor of Clinical Medicine Department ·of Medicine and Institute of Rehabilitation Medicine (Research Assignment) New York University Medical Center, and Assistant Attending Physician"Nephritis Hypertension Clinic Columbia Presbyterian Medical Center New York, New York Ray W. Gifford, Jr., M.D., F.A.C:P., F.A.C.C. Head of the Department of Hypertension and Nephrology Cleveland Clinic Cleveland, Ohio With technical assistance of Mr. Sydney Dufton, Mrs. Mildred Hulse, Mr. Craig J. Hart, Miss Irene von Estorff, and Mr. Thomas W. Rock Designer: Howard Liederman Library of Congress Cataloging in Publication Data Manger, William Muir. Pheochromocytoma. Bibliography: p., Includes index. 1. Pheochromocytoma. I. Gifford, Ray W., Jr., joint author. II. Title. RC280.A3M36 616.1'32 77-8628 All rights reserved. No part of this book may be translated or reproduced in any form without written permission from Springer-Verlag. 9 8 7 6 5 4 3 2 1 © 1977 by Springer-Verlag New York Inc. Softcover reprint of the hardcover 1st edition 1977 ISBN-13 :978-1-4612-9902-8 e- ISBN-13 :978-1-4612-9900-4 DOl: 10.l007/978-1-4612-9900-4 This monograph is dedicated to four doctors whose lives have been so largely given to teaching, encouraging, and inspiring their fellow men. Their compassionate concern for others and their remarkable sensitivity to the needs of both students and associates bring to mind a quotation from Ecclesiasticus (38:2): "For Of The Most High Cometh Healing." Dedication vii outstanding awards and honorary degrees throughout the world. The quality, precision, reliability, and rigid objectivity of his studies have established a mark of excellence revered by all those in­ volved in catecholamine research. Equally im­ pressive is the quality of his character. Despite his fame, he remains unimpre,ssed by his achievements. His calm dignity, sincerity, deep sense of integrity, graciousness, warm hospi­ tality, and deep concern for his fellow men impart a nobility of character rarely observed. His remarkable career and character have set an inspiring example to all those privileged to know him. Albert Schweitzer has said that example is not the most important thing in influencing others; it is the only thing. William Muir Manger Dr. Irvine H. Page has enjoyed a warm friendship with Ulf von Euler for many years. The esteem of one great scientist for another (expressed below by Dr. Page) is · particularly Ulf Svante von Euler appropriate and inspiring. [Physiologist and Teacher) From about 1920 to 1945, a cluster of highly 1905- gifted investigators fashioned a body of knowl­ edge concerned with the neural transmitter of Unquestionably, one of the most accomplished the sympathetic nervous system. It was my and illustrious scientists of our time is Pro­ good fortune to have known almost all of fessor Ulf Svante von Euler. His scientific them personally although their work often genius has produced an incredible array of lay outside my field. There were Cannon, elegant pioneering investigations of extreme Dale, Holtz, and von Euler, to mention only importance to the concepts of sympathetic the most important. It is von Euler to whom nerve function. (It is particularly fitting that this book is dedicated. Professor von Euler be included in the dedica­ I first met Dr. von Euler's father in Munich, tion of this monograph, since he has also Germany, in the late 1920s when I was work­ contributed signifi,cantly to the chemical de­ ing at the Kaiser Wilhelm Institute. A year tection and localization of pheochrorriocy­ or two later it was my good fortune to meet toma and to an understanding of its biochem­ Ulf when we both were working at the Uni­ istry and pathophysiology.) Furthermore, he versity College in London. was the first to identify prostaglandin in hu­ Over the years I have learned that Dr. von man semen. Euler epitomizes two essential qualities of For his contributions in the field of cate­ greatness: humility and the sharp cutting cholamines, including his discovery in 1946 edge of thought. Always, his work has been that the neurotransmitter of the sympathetic thorough and creative-little wonder that the nervous system was norepinephrine, he shared scientific and medical communities have rec­ the Nobel Prize for Physiology in 1970. >II< Pro­ ognized his greatness. In my long journey up fessor von Euler has also served with distinc­ and down the peaks and canyons of this highly tion as chairman of the Nobel Prize Com­ emotive as well as intellectual world, I have mittee for Medicine and as President of the known only a few the equal of Ulf von Euler. Nobel Foundation. He has been honored by His work has spoken for itself and so clearly that it needs no advocacy from me. His hu­ .. It is noteworthy that his father, Hans von Euler, had previously received a Nobel Prize in Chemistry in manity has withstood the test of time-no 1929. small achievement for anyone. viii Dedication capacitation from his illness, he could not perform this service since he felt he was not capable of giving his best effort.) ! The following tribute to Dr. Robert F. Loeb was expressed by Dr. George H. Mc­ Cormack, Jr., a former student who ultimately became Dr. Loeb's personal physician. Robert F. Loeb 1895-1973 " ... whose transmitted effluence cannot die so long as fire ou tlives the parent spark" Adonais, Shelley Each of us differs from his fellow man in a multitude of ways and our common traits are few; fewer still, traits approaching the universal-and their ranks thin fast. As a people, we increasingly resemble William Irwin Thompson's insects, who live out their lives in weeks but pass on acquired informa­ tion to succeeding generations in written records. The winter's generation writes of cold and laughs at the summer's concern with heat. Robert Frederick Loeb The transmitted word thus becomes absurd (Internist and Teacher) and finally perishes, the fire of learning 1895-1973 threatens' never to exceed its parent spark, and we search for someone like Robert F. Anyone fortunate enough to have come into Loeb, who for nearly forty years created com­ intimate contact with Dr. Loeb recognized his mon bonds between students year after year extraordinary brilliance and inflexible integ­ at Columbia's College of Physicians and Sur­ rity. Rarely in a lifetime does one meet a geons and who now, 15 years · after the end human being with such boundless talent. His of his academic career at the university, re­ magnetism, chanri, and style were pure delight mains for all those students and colleagues to his house staff and' professional associates. who knew him, an ideal and an unfailing His inspiration as a teacher created a match­ inspiration. less aura of e~citement and dedication. In One cannot do his memory justice. A list­ 1969 he was cited by Harvard University as ing of his achievements and honors comes no "A man whose career epitomizes both the closer to describing his impact on those around compassion and brilliance of medical science." him than measurement of rainfall tells the As a deeply grateful former student and story of a storm.' house staff officer of Dr. Loeb, I would simply He was Bard Professor of Medicine at say: Columbia University from 1947 to 1960 and He gave us knowledge­ then held the same title Emeritus until his He gave us wisdom­ death; but surely he counted titles as of He gave us direction­ smallest importance. He devoted his life not He gave us flight. to the acquisition of honors, although they William Muir Manger certainly came, but rather to the understand­ (Dr. Loeb, despite illness, had kindly agreed ing of disease, to the relief of suffering, and to critically review this monograph before it to teaching others to carryon the work when reached the publisher. His comments would he had gone; and he achieved each of these have been exceedingly helpful, to say the very goals in a manner and to a degree better than least. Unfortunately, because of further in- did anyone else in his own time and for time Dedication ix yet unknown to come; and he did all this I was privileged to be his student, later without losing personal contact with and a the resident in his department, and, still later, feeling of responsibility for every member of his physician. In the last years of his life he his department~Nobel laureates who worked asked me to take ori that latter role and it is with him and the people who cleaned glass­ most memorable to me that he seemed sur­ ware in his laboratories. prised that I was willing. I know he never He inspired the fiercest of loyalties, and imagined, not for a moment, t~at I valued that, too, lives on, with a th0usand memories that role and service as none other, guarded of things that he did and thought more ably it jealously, and thereby having the chance to than anyone else. He carried out studies that spend time with him to the end, as few out­ led to a successful treatm~nt of Addison's side his family did, got more than I gave~as disease and a rational therapy for the keto­ was usual with the Professor. The memory of acidosis of diabetes mellitus, and in these and that time remains as clear as yesterday and is, other similar areas unfailingly minimized the as everything about him was to me and his importance of his work~and would have been colleagues, unforgettable and rewarding, a dif­ astonished to realize that he had cultivated ferent and more ideal level of experience, a followers who will not let those accomplish­ measure of our later decline, for which there ments be forgotten.