Table Of ContentThe Paraganglionic Chemoreceptor System
Physiology, Pathology, and Clinical Medicine
The Paraganglionic
CheDloreceptor System
Physiology, Pathology, and Clinical Medicine
Frederick G. Zak
Professor of Pathology
Downstate School of Medicine
State University of New York
Brooklyn, New York
Lecturer in Pathology
Mount Sinai School of Medicine
New York, New York
Director of Pathology
Methodist Hospital
Brooklyn, New York
William Lawson
Professor of Otolaryngology
Mount Sinai School of Medicine
New York, New York
Chief of Otolaryngology
Veterans Administration Medical Center
Bronx, New York
Foreword by Vincent j. Hyams
With 225 Illustrations
Springer-Verlag
New York Heidelberg Berlin
Frederick C. Zak, M. D.
Professor of Pathology
Downstate School of Medicine
Brooklyn, N.Y., U.S.A.
William Lawson, M.D., D.D.S.
Professor of Otolaryngology
Mount Sinai School of Medicine
New York, N.Y., U.S.A.
Sponsoring Editors: Wolfgang Bergstedt and Marie Low
Design: Abe Krieger
Production: William J. Gabello
Library of Congress Cataloging in Publication Data
Zak, Frederick G.
The paraganglionic chemoreceptor system.
Bibliography: p.
Includes index.
l. Nonchromaffin paraganglioma. 2. Nonchromaf
fin paraganglia. 3. Chemoreceptors. I. Lawson,
William, 1934- . II. Title. [DNLM: l. Carotid
body. 2. Chemoreceptors. 3. Chromaffin system.
4. Paraganglioma. 5. Paraganglioma, Nonchromaffin.
QZ 380 Z21p]
RC280.N58Z34 616.99'24507 81-13613
AACR2
@ 1982 by Springer-Verlag New York, Inc.
All rights reserved. No part of this book may be translated or reproduced in any form
without written permission from Springer-Verlag, 175 Fifth Avenue, New York, New
York 10010, U.S.A.
The use of general descriptive names, trade names, trademarks, etc. in this publication,
even if the former are not especially identified, is not to be taken as a sign that such
names, as understood by the Trade Marks and Merchandise Marks Act, may accordingly
be used freely by anyone.
Softcover reprint of the hardcover 1st edition 1982
9 8 7 654 3 2 I
ISBN-13: 978-1-4612-5670-0 e-ISBN-13:978-1-4612-5668-7
DOl: 10.1007/978-1-4612-5668-7
To Our First and Most Earnest Teachers, Our Parents
Professor Dr. Emil R. Zak
and Margarete Zak
Alexander Lawson
and Sophia Lawson
Contents
Foreword by Vincent J. Hyams
IX
Preface
Xl
Acknowledgments
Xlll
1 History 1
2 Anatomy and Topography 15
3 Histology 51
4 Histochemistry and Enzymology 61
5 Ultrastructure 85
6 Embryology 133
7 Innervation of the Carotid Body 143
8 Physiology 153
9 Pharmacology, Biochemistry, and Theories of Excitation 189
10 Pathophysiology of the Nonchromaffin Paraganglia 223
11 Tumors: General Considerations 235
12 Tumors: Clinical Behavior 267
13 Carotid Body Tumors 287
14 Glomus Jugulare Tumors 339
15 Vagal Body Tumors 393
16 Laryngeal Body Tumors 413
17 Orbital Tumors 419
Contents
Vlll
18 Tumors of the Nose and Nasopharynx 425
19 Other Craniocervical Tumors 429
20 Thoracic Tumors 435
21 Abdominal Tumors 455
22 Veterinary Tumors 469
References 481
Index 567
Foreword
One would rarely have the opportunity to augment his medical library with a
more exhaustive examination of the subject matter than is contained herein.
Would only that all areas of one's professional responsibilities be available in
such a complete, didactic, written form. Having a decided interest in the subject
of head and neck paragangliomas for the past decade, I have had the opportunity
to explore the subject through published medical literature and personal investi
gation, and feel qualified to commend Doctors Zak and Lawson on their dedica
tion, accuracy, and thoroughness in this presentation. One seeking any particular
aspect of knowledge from historical to an up-to-date concept on the subject
of paragangliomas will find his answer here.
J.
Vincent Hyams
CAPT MC USN
Chairman, Otolaryngic Pathology Department
Armed Forces Institute of Pathology
Washington, D.C.
Preface
One of us (F.G.Z.) initially became interested in the paraganglionic system when
as a resident in pathology at The Mount Sinai Hospital, he performed an autopsy
on an elderly woman with an intracranial tumor of the posterior fossa, which
extended through the jugular foramen into the neck in a dumbbell fashion.
The chief of surgical pathology at that time was Sadao Otani, who 3 years
earlier had recognized histologically a carotid body-like tumor appearing as a
bleeding polyp in the external ear. A quick-frozen section at the time of the
previously mentioned autopsy produced the correct diagnosis. The decalcified
petrous bone showed extensive replacement of marrow spaces by tumor which
projected into the internal jugular vein.
While doing a serial-section study on cancerous larynges, the present authors
discovered homologous structures in typical locations within and outside the
larynx. Neonatal larynges removed at autopsy showed the rather constant occur
rence of these bodies. This work was continued by W.L. with the support and
encouragement of Hugh F. Biller, the Chief of Otolaryngology at The Mount
Sinai Hospital, and culminated in an experimental tissue-culture study of the
chief cells of the normal carotid body and related tumors that proved their
neuroendocrine nature.
The confusion found in the literature concerning the derivation, distribution,
histochemical properties, and function of the normal organ and the observed
differences in the biologic behavior of tumors arising at different sites prompted
the writing of this book. While monographs and textbooks dealing with the
anatomy, physiology, pharmacology, and histochemistry of the normal structures
and reviews of the various tumors have been written, there does not exist a
comprehensive work covering all aspects of the subject. Consequently, this vol
ume was designed to provide this detailed information, as well as to serve as
a bibliographic source book to the world literature.
The anatomy, histology, histochemistry, ultrastructure, physiology, and phar
macology of the paraganglia have been carefully described for workers in each
of these basic science disciplines. The more one delves into the subject, the
xu Preface
more questions appear. While many of the bodies have been identified, many
more exist along the craniosacral outflow of the autonomic nervous system and
await discovery by the astute investigator.
Almost half a century has elapsed since the awarding of the Nobel Prize to
C. Heymans for his discovery of the chemoreceptor nature of these bodies.
However, many of the basic issues regarding chemoreception remain unclear.
The establishment of the element in the organ that is the actual receptor, the
mechanism of chemoreceptor excitation, and the mode of propagation of the
resulting impulse are all problems which are still unsettled despite intense re
search activity throughout the world and several international symposia dedi
cated to these structures.
The concept of single cells or microscopic aggregates distributed throughout
the body and functioning locally as endocrine organs was proposed before the
advent of electron microscopy by Feyrter and Hamperl. A major contribution
occurred when Pearse introduced and later modified the concept of the APUD
or dispersed neuroendocrine system which encompasses cells of similar appear
ance and staining ability which are widely scattered throughout the body of
vertebrates in relation to the respiratory, gastrointestinal, genitourinary, and
other organs. Neoplasms arising from these structures may be hormonally active
when studied with modern immunological methods utilizing purified antisera.
The concept of the carcinoid tumor at whatever location and its modulation
with mucus or melanin production belongs here as well.
The paraganglionic tumors are considered according to their site of origin,
and while they are by no means histologically identical, their biologic behavior
differs widely with the anatomic region. Case reports and clinical data have
been collected from the world literature and the cumulative information analyzed
and collated so as to provide a guide to the diagnosis, treatment, and prognosis
for each group of tumors, based on their natural history locally. Their properties
offamilial tendency, multicentric origin, functional activity, and malignant poten
tial have all been detailed. Their occurrence within the craniocervical region,
principally in relation to the carotid vessels, vagus nerve, temporal bone, larynx,
orbit, nasal cavity, and at intracranial sites, are of importance to the head and
neck surgeon, otolaryngologist, ophthalmologist, and neurosurgeon. The origin
of tumors within the chest and abdomen is also of interest to the thoracic and
general surgeon. The radiographic features of the various neoplasms have been
carefully described and are of value to the diagnostic radiologist. The light
and electron microscopic features of the tumors and their differential diagnosis
are of great importance to the pathologist. A survey of the paraganglionic neo
plasms arising in domestic animals is also of interest to veterinarians. Finally,
review of the role of the chemoreceptors in the regulation of cardiopulmonary
function is indispensable to the anesthesiologist and respiratory physiologist.
A very significant collateral benefit of this book, we believe, is that it provides
a chronologically organized compendium of all important recorded information
on the subject.
Frederick C. Zak
William Lawson
Acknowledgments
We wish to thank Dr. Amiya Patnaik of the Animal Medical Center, New York,
for his assistance with the chapter on veterinary tumors. Certain members of
the staff of the Department of Otolaryngology of the Mount Sinai Medical Center,
New York, have also made invaluable contributions by virtue of their extensive
clinical experience with these neoplasms: Dr. Max L. Som with carotid body
tumors, Dr. Simon Parisier with glomusjugulare tumors, and Dr. Hugh F. Biller
with vagal body and other head and neck chemodectomas. One of us (W.L.)
wishes especially to thank Dr. H. F. Biller, Chairman of the Department of
Otolaryngology, the Mount Sinai Medical School, for his wisdom, friendship,
and support which enabled completion of this book.
We also wish to thank the staff of the Medical Media Services of the Bronx
Veterans Administration Hospital under the direction of Ms. Gloria Spevacek
for their help with drawings, photomicrographs, electronmicrographs, and radio
graphs.
The authors are indebted to Dr. K. Jindrak for his expertise in translating
Czech, Polish, and Russian articles. We are grateful to Mrs. E. Taub, Ms. M.
Lynch, and Mr. J. H. Meece for their excellent librarian help.
We are also indebted to Prof. Max Watzka for furnishing the photograph of
Alfred Kohn, Prof. A. Gallego Fernandez for the photograph of Fernando de
Castro, and Prof. A. F. de Schaepdryver for the photograph of Corneille Hey
mans.
