Adrenal Glands

Dimitrios Linos (Editor)

Jon A. van Heerden (Editor)

Adrenal Glands

Dimitrios Linos ^(Editor) Jon A. van Heerden ^(Editor)

Adrenal Glands

Diagnostic Aspects and Surgical Therapy

With 187 Figures

in 251 Separate Illustrations, Mostly in Color

and 52 Tables

Professor Dr. Dimitrios Linos

Director 1st Surgical Clinic, Hygeia Hospital 227 Kifissias Avenue, 14561 Kifissia, Greece Professor Dr. Jon A. van Heerden

Department of Surgery, Mayo Clinic

First Street SW 200, Rochester, MN 55905, USA

ISBN 3-540-41099-6 Springer-Verlag Berlin Heidelberg New York

Library of Congress Control Number: 2004104845

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Preface

wV d’ eãei¢non men zwn, dia tou¢ton de¢ ãalw¢V zwn.

(Plou¢tarcoV 75 p. C)

Plutarch wrote that Alexander the Great once said that he owed his life to his father but he owed learning how to live well to his teacher, Aristotle.

This book is a tribute to my teacher in surgery Jon van Heerden. “Jon,” for his many friends, yet always

“Dr. van Heerden” for me (who never managed to overcome the anxiety I felt, as a chief resident, when interrupting a golf game to discuss patient problems) has inspired many surgeons worldwide.

It is not accidental that in this book most of the renowned participants have been directly or indirectly students, and/or friends, of Jon van Heerden. The remaining contributors who are not surgeons have known, respected and admired him for his contribu- tion to the field of adrenal surgery.

The magic of JvH, as he is affectionately known in the surgical community, is his keen insight into a com- plicated subject and his ability to transform complex- ity into simplicity. And thus even the complexities of some esoteric adrenal diseases are today readily un- derstood by both generalists and specialists. One of his favorite quotes is in fact:“If it is not awfully simple, it is usually simply awful.”

Skills can be sharpened, knowledge acquired, but what is even more important is the fact that some people transfer their knowledge to others.

It could be argued that greatness in the intellect of a person is only surpassed by the ability to inspire greatness in others; this then is the essence of JvH, who once stated “The greatest satisfaction in surgery lies in the achievements which others are inspired to perform.”

JvH was a Consultant at the Mayo Clinic for 31 years and has influenced and stimulated innumerable trainees to perform the Art of Surgery with integrity and humility. Many, many more surgeons have bene- fited from his devotion to teaching, educating, knowl-

edge sharing, and technical pearls at meetings locally, nationally, and internationally.

As impeccable as he was in his lectures, he was even more meticulous in the operating room. His operating rooms at Mayo were models of decorum, punctuality, expert teamwork, attention to minutiae, the abhor- rence of blood loss, and ultimate respect not only for tissues, but most importantly for the patient. He truly believed in the aphorism:“The most important person in the operating room is the patient – not the surgeon.”

Jon van Heerden was just and understanding but he was not lenient or flexible with members of the oper- ating team and there was no margin for error.

In this book, integrity, skill, validity and reliability are among the qualities the authors have aspired to as they tackle the captivating “mysteries” that shroud the adrenal glands. Precision and concision were strived for in all chapters, which are both up to date and in- formative. With concrete data on the different aspects of adrenal anatomy,physiology and pathology,and the practical and appropriate application of the surgical treatment, the reader can expect to be challenged and enlightened, surprised as well as comforted, and fore- most – educated.

I cannot stress adequately how indebted I feel to the gracious contributions from the world-renowned physicians and surgeons who rose to the challenge of producing this ambitious project with enthusiasm.

Without their contribution this would have never been a possibility, since neither the editors nor the publish- ers would accept anything less than the very best avail- able. A note of special thanks is due to Ms. Stephanie Benko,from Springer-Verlag,and to my assistant Mary Kostopoulou, who were both enthusiastic and tireless in their organizational skills.

Dimitrios A. Linos, MD, FACS

Preface

The medical/surgical world is shrinking due to im- proved and rapid communications and ease of travel.

The contributors to this volume represent many dif- ferent countries, which is most gratifying. We believe that this international flavor will only increase in the future and that this increase is good for all concerned, patients in particular.

We are deeply indebted to all of the contributors to this publication, be they physicians, surgeons, ra- diologists,anesthesiologists,artists,or publishers.They have unselfishly given us and you not only their ex- pertise but their most precious possession – their time.

Lastly, both of us stand on the shoulders of our teachers and our role models, who have enabled us to see a little further than perhaps they did. Humbly, we thus thank Jim Priestley,Bill Remine,Dick Welbourne, Sheldon Sheps, and Bill Young.You, our revered teach- ers, collectively exemplify to us the thoughts elo- quently expressed by Halsted.

It is our sincere wish that you, who have honored us by reading this introduction and what follows, will de- rive a modicum of pleasure as you peruse Adrenal Glands: Diagnostic Aspects and Surgical Therapy. If you per chance read something that may benefit pa- tient care, this educational endeavor will have been well worth the effort and our wishes shall have been granted and fulfilled.

Jon van Heerden You, our readers, are justified to ask “Why is there a

need for yet another textbook on a rare aspect of en- docrine surgery?” We, your editors, albeit biased, feel that the answer is an affirmative one and is based on a number of closely interrelated factors.

Much has changed in the understanding, elucida- tion, and identification and management of almost all aspects of diseases that affect the adrenal glands. We have, indeed, come a long way since Thomas Addison in 1855 succinctly stated “It will hardly be disputed that at the present moment, the function of the supra- renal capsules, and the influence they exercise in the general economy are almost or altogether unknown.”

Similarly, the surgical approach to these retroperi- toneal glands has undergone an amazing revolution in the most recent decade.We wonder if Hugh-Young, fa- mous for developing and popularizing the posterior open approach to the adrenal glands,could have,in his wildest dreams, ever imagined outpatient laparoscop- ic adrenalectomy. We think not!

Today, and in the future, the emphasis is going to be on teamwork with close cooperation and communica- tion between many disciplines when caring for pa- tients with adrenal pathology. Surgeons, in particular, will need to change their daily vocabulary from I to we.

This is an essential factor which this textbook hope-

fully clearly emphasizes and exemplifies, and the var-

ious disciplines are indeed well represented by our

contributors.

Table of Contents

1 History of Adrenal Surgery . . . . 1 Dean A. Harris, Malcolm H. Wheeler

2 Surgical Anatomy . . . . 7 Nancy Dugal Perrier, Michael Sean Boger

3 Hypothalamic-Pituitary-Adrenal Axis . . . . 19 Ioannis Kyrou, Constantine Tsigos

4 The Sympathoadrenal System: Integrative Regulation of the Cortical and the Medullary Adrenal Functions . . . 33 A. Souvatzoglou

5 The Value of Adrenal Imaging

in Adrenal Surgery . . . 41 Bruno Niederle, Gertraud Heinz-Peer,

Klaus Kaczirek, Amir Kurtaran

6 Cushing’s Syndrome . . . 57 Alejandro Ayala, George Chrousos

7 Subclinical Cushing’s Syndrome . . . 67 Felix Beuschlein, Martin Reincke

8 Ectopic Cushing’s Syndrome . . . 75 D. Vassiliadi, S. Tsagarakis, N. Thalassinos

9 Cushing’s Syndrome in Children

and Adolescents . . . 87 George Mastorakos,

Christina Kanaka-Gantenbein

10 Classic Congenital Adrenal Hyperplasia . . . 101 Evangelia Charmandari, George Chrousos, Deborah P. Merke

11 Overview of Mineralocorticoid

Excess Syndromes . . . 115 Richard D. Gordon, Michael Stowasser

12 Primary Aldosteronism:

The Surgical Perspective . . . 127 Bertil Hamberger

13 Primary Adrenocortical Carcinoma . . . 131 Charles A.G. Proye, François N. Pattou,

Jonathan Armstrong

14 Recurrent Adrenocortical Carcinoma . . . . 143 Paolo Miccoli, Pietro Iacconi

15 Metastatic Adrenocortical Carcinoma . . . . 151 D.E. Tsakayannis, Dimitrios A. Linos

16 Virilizing and Feminizing Adrenal Tumors . . 159 Michael Brauckhoff, Oliver Gimm,

Henning Dralle

17 Pheochromocytoma . . . 177 Cord Sturgeon, Quan-Yang Duh

18 Extra-adrenal and Malignant

Pheochromocytoma . . . 189 Gerard M. Doherty

19 Pheochromocytoma in MEN-2A, MEN-2B, and von Hippel-Lindau Disease . . . 195 Geoffrey B. Thompson

20 Benign Paragangliomas . . . 201

Abbie L. Young, William F. Young, Jr.

21 Neuroblastoma . . . 211 Jed G. Nuchtern, Heidi V. Russell,

Jason M. Shohet

22 Miscellaneous Adrenal Neoplasms (Cysts, Myelolipoma, Hemangioma,

Lymphangioma) . . . 223 Melanie L. Richards

23 Adrenal Incidentalomas . . . 231 Miguel F. Herrera, Juan Pablo Pantoja,

Nayví España

24 Clinically Inapparent Adrenal Mass

(Incidentaloma or Adrenaloma) . . . 241 Dimitrios A. Linos

25 Genetic Syndromes Associated

with Adrenal Tumors . . . 251 Göran Åkerström, Per Hellman

26 Adrenal Tumors and Pregnancy . . . 265 M. Nicole Lamb, David R. Farley

27 Adrenal-Sparing Surgery . . . 275 Richard A. Hodin, Antonia E. Stephen

28 Anesthesia for Adrenal Surgery . . . 287 Stavros G. Memtsoudis, Cephas Swamidoss, Maria Psoma

29 Open Left Anterior Adrenalectomy . . . 299 Dimitrios A. Linos

30 Open Right Anterior Adrenalectomy . . . 303 Dimitrios A. Linos

31 Open Posterior Adrenalectomy . . . 309 Dimitrios A. Linos

32 Right Anterior Laparoscopic Adrenalectomy 313 Dimitrios A. Linos

33 Left Anterior Laparoscopic Adrenalectomy . . 321 Dimitrios A. Linos

34 Laparoscopic Lateral Transabdominal

Adrenalectomy . . . 325 Sanziana Roman, Robert Udelsman

35 Posterior Retroperitoneoscopic

Adrenalectomy . . . 333 Martin K. Walz

36 Laparoscopic Partial Adrenalectomy . . . 341 Geoffrey B. Thompson

37 Virtual Reality and Robotic Technologies in Adrenal Surgery . . . 345 Jacques Marescaux, Francesco Rubino,

Luc Soler

Subject Index . . . 355

X

List of Contributors

Åkerström, Göran, MD

Professor, Department of Surgery

Akademiska Sjukhuset, Uppsala Universitet 75185 Uppsala, Sweden

Email: goran.akerstrom@kirurgi.uu.se Armstrong, Jonathan, MD, MB BS, FRACS Priory Thatch, Priory Green

Dunster, Somerset, TA 24 6RY, United Kingdom Email: zoedog68@hotmail.com

Ayala, Alejandro, MD

Pediatric and Reproductive Endocrinology Branch National Institutes of Health

Building 10, Room 9D-42, 10 Center Drive Bethesda, MD 20892, USA

Email: ayalaa@nih.gov Beuschlein, Felix, MD Abteilung Innere Medizin II

Schwerpunkt Endokrinologie & Diabetologie Medizinische Klinik und Poliklinik

Universitätsklinikum Freiburg Hugstetter Strasse 55

79106 Freiburg, Germany

Email: beuschlein@medizin.ukl.uni-freiburg.de Boger, Michael Sean, MD, PharmD

Department of Internal Medicine

Wake Forest University Baptist Medical Center Medical Center Boulevard

Winston-Salem, NC 27157, USA Email: mboger@wfubmc.edu

Brauckhoff, Michael, MD

Klinik für Allgemein-, Viszeral- und Gefäßchirurgie Martin Luther Universität Halle/Wittenberg Ernst-Grube-Strasse 40

06097 Halle/Saale, Germany

Email: michael.brauckhoff@medizin.uni-halle.de Charmandari, Evangelia, MD

Pediatric and Reproductive Endocrinology Branch National Institute of Child Health

and Human Development (NICHD) National Institutes of Health

31 Center Drive, Building 10, Room 9D42 Bethesda, MD 20892-1583, USA

Email: charmane@mail.nih.gov Chrousos, George, MD

Chairman, Department of Pediatrics Agia Sophia University Hospital 125-127 Kifissias Avenue

11524 Ampelokipoi, Athens, Greece Email: chrousog@exchange.nih.gov Doherty, Gerard M., MD

Professor, Section Head, General Surgery A. Alfred Taubman Health Care Center University of Michigan

Room, 2920, 1500 East Medical Center Drive Ann Arbor, MI 48109, USA

Email: gerardd@umich.edu Dralle, Henning, MD

Professor, Klinik für Allgemein-, Viszeral- und Gefäßchirurgie

Martin Luther Universität Halle/Wittenberg Ernst-Grube-Strasse 40

06097 Halle/Saale, Germany

Email: henning.dralle@medizin.uni-halle.de

Duh, Quan-Yang, MD

Professor of Surgery, Surgical Service Veterans Affairs Medical Center University of California 4150 Clement Street

San Francisco, CA 94121, USA Email: quan-yang.duh@med.va.gov España, Nayví, MD

Surgical Resident, Department of Surgery Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán

Vasco de Quiroga 15

Tlalpan 14000, Mexico City, MEXICO Email: nayvi@hotmail.com

Farley, David. R., MD

Professor, Department of Surgery, Mayo Clinic 200 First Street, SW

Rochester, MN 55905, USA Email: farley.david@mayo.edu Gimm, Oliver, MD

Abteilung für Allgemein-, Viszeral- und vaskuläre Chirurgie

Martin Luther Universität Halle/Wittenberg Ernst-Grube-Strasse 40

06097 Halle/Saale, Germany Gordon, Richard D., MD

Professor, Department of Medicine Greenslopes Hospital

4120 Brisbane, Australia

Email: med.greenslopes@ug.edu.au Hamberger, Bertil, MD, PhD, FRCS Professor of Surgery

Department of Surgical Sciences Section of Surgery, Karolinska Hospital 17176 Stockholm, Sweden

Email: bertil.hamberger@kirurgi.ki.se Harris, Dean A., MB, ChB, MRCS

Clinical Research Fellow, Institute of Nephrology College of Medicine, University of Wales

Heath Park

Cardiff, Wales CF14 4XN, United Kingdom

Heinz-Peer, Gertraud, MD Assistant Professor of Radiology Department of Radiology Division of Surgical Radiology Medical University of Vienna Währinger Gürtel 18-20 1090 Wien, Austria

email: gertraud.heinz@meduniwien.ac.at Hellman, Per, MD

Department of Surgical Sciences Universiy Hospital

75185 Uppsala, Sweden

Email: per.hellman@kirurgi.uu.se Herrera, Miguel F., MD, PhD Professor, Department of Surgery Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán

Vasco de Quiroga 15

Tlalpan 1400, Mexico D.F. Mexico Email: herreram@quetzal.innsz.mx Hodin, Richard Aaron, MD

Professor, Chief of Endocrine Surgery Department of Surgery

Massachusetts General Hospital Gray 504, 55 Fruit Street Boston, MA 02114-2696, USA Email: rhodin@partners.org Iacconi, Pietro, MD

Dipartimento di Chirurgia

Facoltà di Medicina e Chirurgia, Università di Pisa Via Roma 67

56126 Pisa, Italy Kaczirek, Klaus, MD Department of Surgery Section of Endocrine Surgery Division of General Surgery Medical University of Vienna Währinger Gürtel 18-20 1090 Wien, Austria

email: klaus.kaczirek@meduniwien.ac.at

XII

Kanaka-Gantenbein, Christina, MD Pediatric Endocrinologist

Unit of Endocrinology, Diabetes and Metabolism First Department of Pediatrics

Agia Sophia Children’s Hospital, University of Athens

Thivon 3 & Mikras Asias Street 11527 Goudi, Athens, Greece Kurtaran, Amir, MD

Associate Professor of Nuclear Medicine Department of Nuclear Medicine Medical University of Vienna Währinger Gürtel 18-20 1090 Wien, Austria

email: amir.kurtaran@meduniwien.ac.at Kyrou, Ioannis, MD

16 Agias Aikaterinis Str.

17352 Agios Dimitrios Athens, Greece

e-mail: kyrouj@otenet.gr Lamb, M. Nicole, MD

Surgical Resident, Morehouse School of Medicine 720 Westview Drive SW

Atlanta, GA 30310-1495, USA Linos, Dimitrios A., MD, Professor

Director of 1st Surgical Clinic, Hygeia Hospital Consultant in Surgey, Massachusetts General Hospital Boston, MA, USA

227 Kifissias Avenue

14561 Kifissia, Athens, Greece Email: dlinos@hol.gr

Email: dlinos@hms.harvard.edu Marescaux, Jacques, MD, FRCS

Chirurgie A, Hôpital Civil, IRCAD-EITS Institute 1 Place de l’Hôpital, BP 426

67091 Strasbourg Cedex, France

Email: Jacques.Marescaux@ircad.u-strasbg.fr Mastorakos, Georgios, MD

3 Neofytou Vamva Str.

10674 Athens, Greece

Email: mastorak@mail.kapatel.gr

Merke, Deborah P.

Chief of Pediatric Services

Clinical Center of the National Institute of Child Health & Human Development (NICHD) 9000 Rockville Pike

Bethesda MD 20892, USA E-mail: merked@mail.nih.gov Memtsoudis, Stavros G., MD Department of Anaesthesiology Preoperative and Pain Medicine Brigham and Women’s Hospital 75 Francis Street

Boston MA 02115, USA

Email: smemtsoudis@comcast.net Miccoli, Paolo, MD

Professor, Dipartimento di Chirurgia

Facoltà di Medicina e Chirurgia, Università di Pisa Via Roma 67

56126 Pisa, Italy

Email: p.miccoli@dc.med.unipi.it Niederle, Bruno, MD, Professor

Professor of Surgery, Department of Surgery Section of Endocrine Surgery

Division of General Surgery Medical University of Vienna Währinger Gürtel 18-20 1090 Wien, Austria

email: bruno.niederle@meduniwien.ac.at Nuchtern, Jed G., MD

Associate Professor of Surgery and Pediatrics Baylor College of Medicine

Texas Children’s Cancer Center 6621 Fannin, CC650.00

Houston, TX 77030, USA Email: nuchtern@bcm.tmc.edu Pantoja, Juan Pablo, MD

Staff Surgeon, Department of Surgery Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán

Vasco de Quiroga 15

Tlalpan 14000, Mexico City, Mexico Email: pantoja@quetzal.innsz.mx

List of Contributors XIII

Pattou, Francois N., MD Assistant Professor of Surgery General Surgical and Endocrine Unit Hospital Huriez

Rue Michel Polonovski 59037 Lille Cedex, France Email: fpattou@univ-lille2.fr Perrier, Nancy Dugal, MD, FACS Associate Professor of Surgery Department of Surgical Oncology

University of Texas, MD Anderson Cancer Center 1400 Holcombe Boulevard

Houston, TX 77230-1402, USA Email: nperrier@mdanderson.org Proye, Charles A.G., MD, Prof.

Service de Chirurgie Générale Endocrinienne Clinique Chirurgicale Est, Hôpital Claude Huriez University de Lille

Rue Michel Polonovski 59037 Lille Cedex, France Psoma, Maria, MD

Anesthesiologist, Hygeia Hospital Consultant in Anesthesia, Mayo Clinic Rochester, MN, USA

Kifissias Avenue & 4 Erythrou Stavrou Street 151 23 Marousi

Athens, Greece

Email: psoma.maria@mayo.edu Reincke, Martin, MD

Professor, Abteilung Innere Medizin II

Medizinische Klinik, Universitätsklinikum Freiburg Hugstetter Strasse 55

79106 Freiburg, Germany

Email: reincke@med1.ukl.uni-freiburg.de Richards, Melanie L., MD

Associate Professor, Department of Surgery University of Texas Health Science Center Room 213E, 7703 Floyed Curl Drive San Antonio, TX 78229-3900, USA Email: richardsm@uthscsa.edu

Roman, Sanziana, MD, FACS Assistant Professor of Surgery

Chief of Endocrine Surgery, Department of Surgery Yale University, School of Medicine

333 Cedar Street

New Haven, CT 06520, USA Email: sanziana.roman@yale.edu Rubino, Francesco, MD

IRCAD-EITS Institute 1 Place de l’Hôpital, BO 426 67091 Strasbourg, France Email: F.rubino@lycos.com Russell, Heidi V., MD

Assistant Professor of Pediatrics Baylor College of Medicine Texas Children’s Cancer Center 6621 Fannin

Houston, TX 77030, USA Email: hvrussell@txccc.org Shohet, Jason M., MD, PhD Assistant Professor of Pediatrics Baylor College of Medicine Texas Children’s Cancer Center 6621 Fannin

Houston, TX 77030, USA Email: jshohetbcm.tmc.edu Soler, Luc, PhD

IRCAD-EITS Institute 1 Place de l’Hôpital, BP 426 67091 Strasbourg, France email: luc.soler@ircad.u-strasb.fr Souvatzoglou, Athanasios, MD 92 Vassilis Sofias Avenue 11528 Athens, Greece

email: dkpim@ath.forthnet.gr Stephen, Antonia E.

Division of Surgical Oncology Massachusetts General Hospital 55 Fruit Street

Boston, MA 02114, USA Email: astephen@partners.org

XIV

Stowasser, Michael, PhD, FRACP Associate Professor, Hypertension Unit Princess Alexandra Hospital

Wooloongabba

4120 Brisbane, Australia Email: mstowasser@ug.edu.au Sturgeon, Cord, MD

Department of Surgery

Veterans Affairs Medical Center University of California 4150 Clement Street

San Francisco, CA 94121, USA Swamidoss, Cephas, MD Department of Anesthesiology New York-Presbyterian Hospital Weill-Cornell Medical College 525 East 68th Street, Floor G3 New York, NY 10021, USA Thalassinos, Nikolas, MD Professor

24 Ravine Street 11521 Athens, Greece

Email: endo.evangel@acisgroup.gr Thompson, Geoffrey B., MD Professor, Department of Surgery Mayo Clinic and Mayo Foundation 200 First Street SW

Rochester, MN 55905, USA

Email: thompson.geoffrey@mayo.edu Tsagarakis, S., MD

Department of Endocrinology

Diabetes and Metabolism, Evangelismos Hospital 45-47 Ipsilantou Street

10676 Kolonaki Athens, Greece

Tsakayannis, Dimitrios E., MD 16 Karneadou Street

10675 Athens, Greece Email: dtsak@ath.forthnet.gr

Tsigos, Constantine, MD

Professor, Hellenic National Center for Research, Prevention and Treatment of Diabetes Mellitus and Its Complications

3, Ploutarchou Street 10675 Athens, Greece Email: ctsigos@hndc.gr Udelsman, Robert, MD, MBA

Professor, Chairman, Department of Surgery Yale-New Haven Hospital

330 Cedar Street, FMB 102 New Haven, CT 06520, USA Email: robert.udelsman@yale.edu Vassiliadi, D., MD

Department of Endocrinology

Diabetes and Metabolism, Evangelismos Hospital 45-47 Ipsilantou Str.

10676 Kolonaki Athens, Greece Walz, Martin K., MD

Professor, Abteilung für Chirurgie Zentrum für Minimalinvasive Chirurgie Kliniken Essen-Mitte

Henricistrasse 92 45136 Essen, Germany

Email: martin.walz@uni-essen.de Email: mkwalz@kliniken-essen-mitte.de Wheeler, Malcom H., MD

Professor, Department of Endocrine Surgery University Hospital of Wales

Heath Park

Cardiff CF4 4XW, United Kingdom Email: M.H.Wheeler@btinternet.com Young, Abbie L., MS

Professor, Genetic Counselor Minnesota Department of Health 717 Delaware Street SE

Minneapolis, MN 55440, USA

email: Abbie.Young@health.state.mn.us

Young, William F., Jr. MD, MSc, FACP, FRCP (Edin) Professor of Medicine, Division of Endocrinology and Metabolism and Nutrition, Mayo Clinic 200 First Street S.W.

Rochester, MN 55905, USA email: Young.William@Mayo.edu

List of Contributors XV