MANAGEMENT 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