Table Of ContentMANAGEMENT OF CHILDHOOD BRAIN TUMORS
Foundations o/Neurological Surgery
Michael Salcman, Series editor
1. Robertson and Clark, Lasers in Neurosurgery.
ISBN 0-89838-966-6.
2. AI-Mefty, Surgery of the Cranial Base. ISBN 0-89838-418-4.
3. Deutsch, Management of Childhood Brain Tumors.
ISBN 0-7923-0669-4.
MANAGEMENT OF
CHILDHOOD BRAIN
TUMORS
Edited by
Melvin Deutsch, M.D.
University Health Center of Pittsburgh
"~.
Kluwer Academic Publishers
BOSTON DORDRECHT LONDON
DISTRIB UTORS
For North America: Kluwer Academic Publishers, 101 Philip Drive, Assinippi Park,
Norwell, Massachusetts 02061 USA
For all other countries: Kluwer Academic Publishers Group, Distribution Centre,
Post Office Box 322,3300 AH Dordrecht, THE NETHERLANDS
Library of Congress Cataloging-in-Publication Data
Management of childhood brain tumors 1 edited by Melvin Deutsch.
p. cm. - (Foundations of neurological surgery; FONS3)
ISBN-13: 978-1-4612-8807-7 e-ISBN-13: 978-1-4613-1501-8
DOl: 10.1007/978-1-4613-1501-8
l. Brain-Tumors. 2. Tumors in children. I. Deutsch, Melvin.
II. Series.
[DNLM: 1. Brain Neoplasms-in infancy & childhood. 2. Brain
Neoplasms-therapy. WL 358 M266}
RC280.B7M36 1990
DNLM/DLC
for Library of Congress 90-4062
ClP
COPYRIGHT
© 1990 by Kluwer Academic Publishers
Softcover reprint of the hardcover 1s t edition 1990
All rights reserved. No part of this publication may be reproduced, stored in a retrieval
system or transmitted in any form or by any means, mechanical, photocopying, recording,
or otherwise, without the prior written permission of the publisher, Kluwer Academic
Publishers, 101 Philip Drive, Assinippi Park, Norwell, Massachusetts 0206l.
To Lisa and Sara
CONTENTS
Contributing Authors lX
Preface Xl
1. Introduction 1 13. Supratentorial Primitive Neuroectodermal
Melvin Deutsch Tumors 343
Paul M. Zeltzer, Sarah]. Gaskill, Arthur E. Marlin
2. Tumors of the Central Nervous System
in Children 5 14. Meningioma 357
Laurence E. Becker, Venita Jay Melvin Deutsch
3. Diagnostic Radiology of Childhood Brain 15. Ependymomas 369
Tumors 53 Craig L. Silverman, Patrick R.M. Thomas,
David W. Johnson, Richard E. Latchaw William Cox
4. Neurological Assessment 121 16. Intracranial Germ-Cell Tumors 383
Jerrold M. Milstein Melvin Deutsch
5. Endocrine and Systemic Manifestation 17. Midline Intra-axial Tumors (Nuclear and
of Brain Tumors in Children 13 7 Brainstem) 401
Silva Arslanian, ThomasP. Foley, Jr. , PeterA. Lee A. Leland Albright
6. Surgery 175 18. Medulloblastoma 411
A. Leland Albright Melvin Deutsch
7. Radiotherapy 187 19. Cerebellar Astrocytomas 441
Melvin Deutsch Harold]. Hoffman, Mitchell S. Berger, Laurence E.
Becker
8. Pathophysiologic Basis for Brain Tumor
20. Brain Metastases 457
Chemotherapy 233
Jeffrey C. Allen
Jeffrey A. Stephenson, Jonathon L. Finlay
21. Recurrent Central Nervous System
9. Visual Pathway Gliomas 273
Tumors 465
Roger J. Packer, Peter]. Savino, Gary R. Diamond,
Leslie N. Sutton Melvin Deutsch
10. Craniopharyngiomas 285 22. Late Sequelae in Survivors of Childhood
Brain Tumors 481
Dachling Pang
Melvin Deutsch
11. Pituitary Adenomas in Childhood 309
Dachling Pang 23. Long-term Neuropsychologic and
Intellectual Sequelae in Brain Tumor
12. Cerebral Hemisphere Glioma 325 Patients 493
Melvin Deutsch Craig L. Silverman, Patrick R.M. Thomas
vii
Vll1
24. Second Primary Tumors in Treated
Patients 501
Melvin Deutsch
Addendum to Chapter 2: Tumors of the central
nervous system in children 511
Index 517
CONTRIBUTING AUTHORS
A. Leland Albright, M.D. Gary R. Diamond, M.D.
Associate Professor, Neurosurgery Associate Professor, Ophthalmology
University of Pittsburgh Hahnemann University
School of Medicine School of Medicine
Pittsburgh, PA 15213 Broad and Vine Streets
Philladephia, PA 19102-1192
Jeffrey C. Allen, M.D.
Associate Professor,
Pediatrics and Neurology
Jonathon L. Finlay, M.D.
Departments of Neurology and Pediatrics
Associate Professor, Pediatrics
New York University Medical Center
Vice Chairman, Department of Pediatrics
550 First Avenue
Memorial Sloan Kettering Hospital
New York, NY 10016 New York, NY
Silva Arslanian, M.D.
Assistant Professor, Pediatrics Thomas P. Foley, Jr., M.D.
Children's Hospital Professor, Pediatrics
University of Pittsburgh University of Pittsburgh
School of Medicine School of Medicine
Pittsburgh, PA 15213 Chief, Division of Endocrinology
Children's Hospital
Laurence E. Becker, M.D., F.R.C.P. (C)
Professor, Pathology Pittsburgh, PA 15213
Head, Division of Neuropathology
University of Toronto Sarah J. Gaskill, M. D.
Faculty of Medicine Resident, Neurosurgery
The Hospital for Sick Children Department of Neurosurgery
Toronto, Ontario, Canada Duke University
Mitchell S. Berger, M.D. Durham, NC 22706
Assistant Professor, Neurosurgery
University of Washington HaroldJ. Hoffman, M.D., F.A.C.S. (C)
Medical Center F.A.C.S.
School of Medicine Professor, Surgery
1959 N.E. Pacific Avenue Division of Neurosurgery
Seattle, WA 98195 University of Toronto
Faculty of Medicine
William Cox, M. D.
Hospital for Sick Children
Professor, Neurosurgery
Toronto, Ontario, Canada
Washington University
School of Medicine
Venita Jay, M.D., F.R.C.P.C.
660 South Euclid Avenue
Assistant Professor, Pathology
St. Louis, MO 63110
Department of Pathology
Melvin Deutsch, M.D., F.A.C.R. University of Toronto
Professor, Radiation Oncology Faculty of Medicine
University of Pittsburgh Neuropathologist
School of Medicine Hospital for Sick Children
Pittsburgh, PA 15213 Toronto, Ontario, Canada
ix
x
David W. Johnson, M.D. Dachling Pang, M.D., F.R.C.S. (C), F.A.C.S.
Assistant Professor, Radiology Associate Professor, Neurosurgery
University of Pittsburgh University of Pittsburgh
School of Medicine School of Medicine
Pittsburgh, PA 15213 Chief, Pediatric Neurosurgery
Children's Hospital
Richard E. Latchaw, M.D., F.A.C.R. Pittsburgh, PA 15213
Department of Radiology
Peter]. Savino, M.D.
Swedish Hospital
Clinical Professor, Ophthalmology
Ingelwood, Colorado
University of Pennsylvania
Formerly Professor and Chairman
School of Medicine
Department of Diagnostic Radiology
Thomas Jefferson University
University of Pittsburgh
School of Medicine
School of Medicine
Chief, Neuro-Ophthalmology
Pittsburgh, PA 15213
Willis Eye Hospital
Philadelphia, PA 19104
Peter A. Lee, M.D., Ph.D. Craig 1. Silverman, M.D.
Professor, Pediatrics Assistant Professor, Radiology
University of Pittsburgh University of Temple Hospital
School of Medicine Philadelphia, PA 19104
Children's Hospital of Pittsburgh
Jeffrey A. Stevenson, M.D.
Pittsburgh, PA 15213
Adjunct Clinical Professor of Radiation
Oncology
East Carolina University School of Medicine
Arthur E. Marlin, M.D.
Director of Radiation Oncology
Associate Clinical Professor, Orthopaedics and
Mercy Hospital ofJ anesville
Pediatrics
1000 Mineral Point Avenue
University of Texas
Janesville, WI 53545
Health Science Center
7703 Floyd Cud Drive
Lesley N. Sutton, M.D.
San Antonio TX 78284-7790
Associate Professor Neurosurgery and Pediatrics
University of Pennsylvania
School of Medicine
Jerrold M. Milstein, M.D.
Philadelphia, PA 19104
Associate Professor, Pediatrics and Medicine
(Neurology) Patrick R.M. Thomas, M.B.B.S.
Children's Hospital and Medical Center Professor, Radiology
University of Washington Washington University
Seattle, WA 98195 School of Medicine
660 South Euclid Avenue
St. Louis, MO 63110
Roger]. Packer, M.D.
Professor, Neurology and Pediatrics Paul M. ZeItzer, M.D.
George Washington University Associate Professor, Pediatrics
Director, Department of Neurology University of Southern California
Children's National Medical Center Director, Brain Tumor Program
III Michigan Avenue, NW Children's Hospital of Los Angeles
Washington, DC 20010 Los Angeles, CA 90033
PREFACE
In children, the central nervous system tumors complished through advances in the three main
are exceeded in incidence only by leukemia and antineoplastic therapeutic modalities-surgery,
are more common than any of the other malig radiation therapy, and chemotherapy. Improve
nancies of childhood. Childhood central nervous ments in neurosurgical technique concomitant
system tumors encompass a range of histo with improvements in anesthesia and periopera
logic varieties from the histologically benign tive supportive care have resulted in decreased
appearing pilocytic astrocytoma to the extremely morbidity and mortality from neurosurgical
malignant-appearing glioblastoma multiforme procedures together with an increased likelihood
and the undifferentiated primitive neuroecto of accomplishing a gross total resection of
dermal tumors. Similarly, the biologic behavior tumor. Radiotherapy has evolved from using
of childhood brain tumors varies not only ac orthovoltage (200-250 KV) equipment to
cording to histology but also with location of supervoltage equipment with much-improved
the tumor and age. Unlike primary central nerv penetration, thus allowing for the administra
ous system tumors in adults, many varieties of tion of accurate homogeneous high doses to
childhood brain tumors have the propensity to large volumes without significant effects on the
disseminate via the cerebrospinal fluid path overlying skin and soft tissues. Preliminary data
ways. With improvements in length of survival, indicate that multiagent chemotherapy as ad
dissemination beyond the central nervous sys juvant therapy may be increasing the long-term
tem is becoming a more common occurrence, survival rates of children afflicted with supraten
especially for medulloblastoma. torial malignant glioma and posterior fossa
Improvements in therapeutic outcome for medulloblastoma. Undoubtedly, the advent of
adult primary malignant glioma over the past computerized tomography and now magnetic
two to three decades can be measured by a few resonance imaging has contributed significantly
months increase in median survival. In children, to the management of the child with a brain
remarkable advances have been made in the treat tumor; diagnosis of intracranial tumor and
ment of brain tumors. In the first half of this hydrocephalus is now more accurately obtained
century, the only afflicted children likely to without doing invasive, potentially dangerous
survive were those with histologically and bio procedures, and thus earlier diagnosis of tumor
logically nonaggressive tumors amenable to is possible. These imaging techniques also have
complete resection, such as cerebellar astro allowed for a much more precise location of
cytomas. Many tumor types that previously tumor for radiotherapy planning than was poss
were almost 100% lethal, such as medullo ible in prior times, when nucleotide scanning,
blastoma and supratentorial malignant astro angiography, and pneumoencephalography were
cytoma, can now be effectively treated, resulting used. Likewise, with these new imaging modal
in long-term survival for about half of all pa ities, posttherapy evaluation is much easier and
tients. However, some tumors, such as brain more accurate, allowing for determination of the
stem gliomas, still have a dismal outlook that amount of postresection residual tumor, surgi
has not changed appreciably over the past cally related complications such as hemorrhage,
several decades. shunt malfunction, and recurrent tumor.
Improvements in outcome have been ac- Along with the improvements in long-term
Xl
