Tumors of the Pediatric Central Nervous System Second Edition Tumors of the Pediatric Central Nervous System Second Edition Edited by Robert F. Keating, MD Professor and Chief Department of Neurosurgery Children’s National Medical Center George Washington School of Medicine Washington, DC James Tait Goodrich, MD, PhD Professor of Clinical Neurological Surgery, Clinical Pediatrics, and Clinical Surgery Leo M. Davidoff Department of Neurological Surgery Montefiore Medical Center Bronx, New York Roger J. Packer, MD Senior Vice President Center for Neuroscience and Behavioral Medicine Director, Brain Tumor Institute Director, Daniel and Jennifer Gilbert Neurofibromatosis Institute Investigator, Center for Neuroscience Research (CNR) Children’s National Medical Center Washington, DC Thieme New York • Stuttgart Thieme Medical Publishers, Inc. 333 Seventh Ave. New York, NY 10001 Executive Editor: Kay Conerly Managing Editor: Judith Tomat Editorial Assistant: Christine Crigler Senior Vice President, Editorial and Electronic Product Development: Cornelia Schulze Production Editor: Barbara A. Chernow International Production Director: Andreas Schabert Vice President, Finance and Accounts: Sarah Vanderbilt President: Brian D. Scanlan Compositor: Carol Pierson, Chernow Editorial Services, Inc. Printer: Everbest Printing Co. Library of Congress Cataloging-in-Publication Data Tumors of the pediatric central nervous system I [edited by] Robert F. Keating, James Tait Goodrich, Roger J. Packer. — 2nd ed. p. ; cm. Includes bibliographical references and index. ISBN 978-1-60406-546-6 — ISBN 978-1-60406-547-3 (eiSBN) I. Keating, Robert F. II. Goodrich, James T. III. Packer, Roger J., 1951– [DNLM: 1. Central Nervous System Neoplasms—therapy. 2. Child. 3. Infant. WL 358) 618.92′9948—dc23 2012036153 Copyright ©2013 by Thieme Medical Publishers, Inc. This book, including all parts thereof, is legally protected by copyright. Any use, ex- ploitation, or commercialization outside the narrow limits set by copyright legislation without the publisher’s consent is illegal and liable to prosecution. This applies in particular to photostat reproduction, copying, mimeographing or duplication of any kind, translating, prepa- ration of microfilms, and electronic data processing and storage. Important note: Medical knowledge is ever-changing. As new research and clinical experience broaden our knowledge, changes in treat- ment and drug therapy may be required. 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Keating To our teachers, colleagues, and ultimately our patients and their families; we remain indebted to them for their patience, trust, and gratitude after untold sleepless nights fraught with inexorable discomfort. We answer their call to do our best and offer a light at the end of their tunnel. As always, none of this would have been possible without the undying love and support from my wife, Susan, and my sons, Douglas and Gregory. James Tait Goodrich In infinito vacuo, ex fortuitâ atomorum collisione! [Fortunately the gods have allowed me more than just one fortunate collision!] Reflecting back over a career in education, I have often thought back on the teachers who have had the most influence on me. Recognizing that this book has been put together by a group of educators, myself included, I thought it appropriate to dedicate my portion to a group of educators who have had a special influence on my life: Donald Collins, Professor of Biological Sciences, Orange Coast College A natural educator, one who was able to instill in a young and rather immature individual’s mind the love for science and biology. You provided the first and most significant spark in a high school dropout; you ignited the flame, and its brilliance has never died! James L. McGaugh, Professor of Psychobiology, University of California at Irvine I came to UCI fresh out of a community college. I spent many pleasurable hours in your lab studying the intricacies of learning and memory. I learned from you that to be an educator one needs to not only know a subject well, one must be able to deliver it in a fashion that all can understand—of this you are truly the master! Paul L. Kornblith, former Chairman of Neurosurgery, Albert Einstein College of Medicine, Montefiore Medical Center, Bronx, New York As a young, graduating resident of a neurosurgery program I had little idea of where to go or what field to pursue in neuro surgery. My initial thoughts were vascular neurosurgery. At a counter in a small fish restaurant in Washington, DC, you said, “pediatric neurosurgery is where it is at”—wonderful foresight on your part. For during the following five years we developed a most remarkable and vibrant program at Montefiore. What wonderful and halcyon days they were! Judy L. Goodrich, Wife and Mentor In December of 1968, I was discharged from the U.S. Marine Corps after having completed a tour of duty in Vietnam. My only goal at that point was hedonism—to find the finest wave on the Southern California coast and end the day with a great bottle of wine (albeit one with a screw top). I did all this well, and then I came to the point when I had to wake up to reality. W hatever made you decide to join me on this voyage I will never know. You have been a very special and constant companion, a unique person in your understanding of the complexities of this job that I have. After 30-plus years, you are truly my greatest treasure and mentor. Thanks for always being there. Roger J. Packer I would like to thank my wife, Bernice, my children, Zehava and Michael, their spouses (Ophir and Rachel), and our newly minted granddaughter, Anabel, for their love and support. Thanks also to my wonderful colleagues in neurosurgery, neurol- ogy, oncology, neuroradiology, neuropathology, neuropsychology, and nursing for all their help and expertise. It really takes a village. Finally, thanks to all the patients and their families, for honoring me and our team at Children’s National with their trust. I increasingly marvel at their strength and ability to continue to fight with such dignity, love, and endless devotion. Contents Foreword . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiii Section I . Current Therapeutic Strategies 1. Pediatric Neuro-Oncology: A Historical Perspective, James Tait Goodrich.............................................. 3 2. Epidemiology, Greta Bunin ........................................................................................ 27 3. Neurodiagnostic Principles, Courtney A. Lyle and John R. Crawford.................................................... 32 4. Neuroradiology, Gilbert Vézina and Timothy N. Booth ................................................................ 39 5. Neuropathology, Mariarita Santi and Elisabeth J. Rushing ............................................................ 63 6. Cytogenetic/Molecular Biology: Recent Advances in the Cytogenetics and Molecular Biology of Pediatric Brain Tumors, Jong Hee Chang, Yuzo Terakawa, Christian A. Smith, and James T. Rutka.......................... 69 7. Inheritable Genetic Variations Predisposing to CNS Tumors, Jonathan L. Finlay and Uri Tabori........................... 80 8. Anesthesia Considerations, Thalia C. Palmer ........................................................................ 104 9. Surgical Strategies and the Pursuit of Minimalism, Robert F. Keating .................................................. 109 10. Critical Care, Nathan Dean ........................................................................................ 116 11. Radiation Therapy for CNS Malignancies, Shannon M. MacDonald and Nancy J. Tarbell.................................. 138 12. Stereotactic Radiosurgery, Amanda L. Yaun ......................................................................... 146 13. Convection-Enhanced Delivery, Gautam U. Mehta and Russell R. Lonser ............................................... 153 14. Chemotherapy, Eugene I. Hwang, Lindsay B. Kilburn, and Brian R. Rood................................................ 159 15. Gene Therapy for Pediatric Brain Tumors, Kaveh Asadi-Moghaddam, E. Antonio Choicca, David W. Pincus, and Corey Raffel ................................................................ 167 Section II . Tumors Subsection A—Supratentorial 16. Supratentorial Astrocytomas, Ian F. Pollack ......................................................................... 181 17. Optic/Visual Pathway Gliomas, Robert A. Avery, John S. Myseros, and Roger J. Packer.................................... 188 18. Supratentorial Ependymomas, Michael DeCuypere, Paul Klimo Jr., Robert A. Sanford, and Frederick A. Boop ............... 197 19. Craniopharyngiomas, Jeffrey H. Wisoff and Robert E. Elliott........................................................... 205 20. Supratentorial Primitive Neuroectodermal Tumors, Sunil Bhat, Verity Ann Ahern, and Stewart J. Kellie ................... 216 vii viii Contents 21. Pituitary Tumors, Erin N. Kiehna, Edward R. Laws Jr., and John A. Jane Jr................................................ 230 22. Pineal Region Tumors, Muhammad M. Abd-El-Barr, Kristopher G. Hooten, Gregory J.A. Murad, Anthony T. Yachnis, Jeffrey Alan Bennett, Amy Smith, and David W. Pincus.............................................. 237 23. Molecular Biology of Neurofibromatosis, Giorgio Perilongo........................................................... 255 24. Choroid Plexus Tumors, Richard G. Ellenbogen and Daniel J. Donovan.................................................. 265 25. Meningiomas, John S. Myseros and Robert F. Keating . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 276 26. Oligodendrogliomas, Simon J. Hanft, Randy D’Amico, and Richard C.E. Anderson ........................................ 284 27. Supratentorial Gangliogliomas, Mark Fedor and Mark D. Krieger...................................................... 296 28. Infantile Malignant Brain Tumors, Katja von Hoff and Stefan Rutkowski ............................................... 303 29. Dysembryoplastic Neuroepithelial Tumors, Tene A. Cage, Tarik Tihan, and Nalin Gupta ................................. 313 30. Pleomorphic Xanthoastrocytoma, Andrew M. Bauer and Bermans J. Iskandar .......................................... 319 31. Uncommon Supratentorial Brain Tumors, Amanda Muhs Saratsis and Suresh N. Magge ................................. 324 32. Extraneural Metastasis, David F. Jimenez and John R. Floyd II ......................................................... 336 Subsection B—Brainstem 33. Brainstem Gliomas, Amanda Muhs Saratsis, Javad Nazarian, and Suresh N. Magge...................................... 347 Subsection C—Infratentorial 34. Infratentorial Astrocytomas, Sandi Lam and Ian F. Pollack............................................................ 357 35. Medulloblastomas, Jacques Grill and Christelle Dufour ............................................................... 364 36. Atypical Teratoid/Rhabdoid Tumors, Alyssa Terry Reddy ............................................................. 374 37. Infratentorial Ependymomas, Paul Klimo Jr., Michael DeCuypere, Robert A. Sanford, and Frederick A. Boop ................ 380 38. Miscellaneous Infratentorial Tumors, John S. Myseros................................................................ 389 Subsection D—Skull Base 39. Chordomas, Esthesioneuroblastomas, and Orbital Metastases, Derek Andrew Bruce.................................... 401 Subsection E—Spinal Cord 40. Intramedullary Spinal Cord Astrocytomas, Shlomi Constantini, Akiva Korn, Zvi Lidar, and Cheryl A. Muszynski............ 411 41. Spinal Ependymomas, Chetan Bettegowda, Anubhav G. Amin, and George I. Jallo ....................................... 423 42. Gangliogliomas and Miscellaneous Spinal Cord Tumors, John S. Myseros and Robert F. Keating .......................... 432 43. Neurofibromatosis: A Clinical Overview, James Tait Goodrich and Robert W. Marion.................................... 445 Section III . Outcome and Future Directions 44. Postoperative Considerations, Michael E. Seiff, Kelly Schmidt, and James Tait Goodrich .................................. 469 45. Cooperative Group Trials for Brain Tumors: Organization and Current Directions, Roger J. Packer, Richard Sposto, David A. Walker, and Giorgio Perilongo............................................................... 493 46. Postchemotherapy Morbidity, Gary Nicolin and Colin Kennedy ....................................................... 505 47. Posttherapy Neurologic Sequelae, Elizabeth M. Wells ................................................................ 514 48. Cognitive Considerations, Karin S. Walsh ........................................................................... 523 49. Second Tumors of the Central Nervous System, Joseph P. Neglia....................................................... 531 Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 537