M. D. ANDERSON CANCER CARE S E R I E S Series Editors Aman U. Buzdar, MD Ralph S. Freedman, MD, PhD M. D. ANDERSON CANCER CARE SERIES Series Editors: Aman U. Buzdar, MD, and Ralph S. Freedman, MD, PhD K.K. Hunt, G.L. Robb, E.A. Strom, and N.T. Ueno, Eds., Breast Cancer F.V. Fossella, R. Komaki, and J.B. Putnam, Jr., Eds., Lung Cancer J.A. Ajani, S.A. Curley, N.A. Janjan, and P.M. Lynch, Eds., Gastrointestinal Cancer K.W. Chan and R.B. Raney, Jr., Eds., Pediatric Oncology P.J. Eifel, D.M. Gershenson, John J. Kavanagh, and Elvio G. Silva, Eds., Gynecologic Cancer F. DeMonte, M.R. Gilbert, A. Mahajan, and I.E. McCutcheon, Eds., Tumors of the Brain and Spine Franco DeMonte, MD, Mark R. Gilbert, MD, Anita Mahajan, MD, and Ian E. McCutcheon, MD Editors The University of Texas M. D. Anderson Cancer Center, Houston, Texas Tumors of the Brain and Spine Foreword by Raymond Sawaya, MD, and W. K. Alfred Yung, MD With 73 Illustrations and 32 Tables Franco DeMonte, MD Mark R. Gilbert, MD The University of Texas The University of Texas Department of Neurosurgery Department of Neuro-Oncology M. D. Anderson Cancer Center M. D. Anderson Cancer Center 1515 Holcombe Blvd., Unit 442 1515 Holcombe Blvd., Unit 431 Houston, TX 77030-4009, USA Houston, TX 77030-4009, USA Anita Mahajan, MD Ian E. McCutcheon, MD The University of Texas The University of Texas Department of Radiation Oncology Department of Neurosurgery M. D. Anderson Cancer Center M. D. Anderson Cancer Center 1515 Holcombe Blvd., Unit 97 1515 Holcombe Blvd., Unit 442 Houston, TX 77030-4009, USA Houston, TX 77030-4009, USA Series Editors: Aman U. Buzdar, MD Ralph S. Freedman, MD, PhD Department of Breast Medical Oncology Department of Gynecologic Oncology The University of Texas The University of Texas M. D. Anderson Cancer Center M. D. Anderson Cancer Center Houston, TX 77030-4009, USA Houston, TX 77030-4009, USA TUMORS OF THE BRAIN AND SPINE Library of Congress Control Number: 2006939206 ISBN-13: 978-0387-29201-4 e-ISBN-13: 978-0387-29202-1 Printed on acid-free paper. © 2007 Springer Science+Business Media, LLC All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, LLC, 233 Spring Street, New York, NY10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed in the United States of America. 9 8 7 6 5 4 3 2 1 springer.com F OREWORD It is frequently stated that progress in the management of tumors of the brain and spine has not occurred in the past 25 years. Such statements seriously underestimate and misrepresent progress in managing various central nervous system tumors, the tremendous technologic enhancements that have revolutionized the imaging of the tumors and the host organ, the multitude of surgical adjuncts such as computerized imaging guidance and functional mapping of the brain and its tracts that are routinely used in the modern neurosurgical operating room, and the highly conformal delivery of radiation to the tumor mass with remarkable sparing of the surrounding nervous tissue. Underlying these technologic improvements are the changes in philosophy that have resulted in a true multidisciplinary approach. Equally significant is the molecular revolution that is identifying key markers behind the genesis of brain tumors, their proliferation, and their resistance to therapy. These molecular discoveries have ushered in the era of molecular targeted therapy, as first demonstrated by the extraordinary success story in chronic myelogenous leukemia of imatinib (Gleevec). The rational combination of targeted agents will revolutionize the way chemo- therapy is given, either alone or in conjunction with radiotherapy. Lastly, focused attention to patients’ well-being has emphasized all measures of quality of life, has encouraged greater participation by patients and their caregivers in the decision-making process, and has encouraged the development of a variety of quality-of-life measurement tools to ensure that adverse effects of treatment are recognized and addressed. In this monograph of the M. D. Anderson Cancer Care Series, the authors have captured the essence of the progress experienced to date and, in the most appropriate multidisciplinary fashion, they have outlined the principles behind patient assessment, decision making, and management of tumors of the brain and spine. Their expertise and commitment to their patients are evident throughout. Raymond Sawaya, MD W. K. Alfred Yung, MD P REFACE Although the annual incidence of intrinsic tumors of the central nervous system (CNS)—about 17,000 to 20,000 in the United States—is much lower than that of more common cancers arising in the lung, breast, or other sites, CNS tumors are prominent in oncology for several reasons. First, they attack the very structure of our personhood and in so doing, create fear and functional deficits as profound as they are disturbing to patients and their families alike. Second, CNS tumors can be difficult to cure when they are infiltrative or located in places that are difficult to access surgically without putting patients at some risk. Third, since tumors originating in any organ system can secondarily affect the brain or spine, much crossover exists between neuro-oncology and general oncology. And finally, CNS tumors are prominent in oncology because of the great strides being made in our understanding of these tumors on a molecular and genetic level and because treatments can now be based on hitherto unrecognized genetic alterations, advances that go hand in hand with similar knowledge being gathered in all the subspecialties of oncology. Because of our rapidly increasing knowledge about tumors of the brain and spine, these tumors are becoming more treatable—either for palliation or for cure—than ever before. The effects of environmental exposures on tumor formation are coming more into focus, and epidemiologic knowledge is now linking nicely with molecular genetic alterations derived from kindreds susceptible to brain tumor formation. Such alterations have been correlated with a progression from benign to malignant forms of several tumor types, most notably the astrocytoma; and although an initiating genetic mutation cannot yet be traced for most tumors, molecular genetics is now being used in neuropathologic diagnoses to supplement more tra- ditional histologic approaches. Diagnostic tools such as magnetic resonance imaging continue to develop and have become quite sensitive, albeit less than perfectly specific, in revealing CNS tumors at earlier and earlier stages. Surgical procedures too are advancing through a combination of more complex technologies and the development of a cadre of neurosurgeons specialized in the nuances of tumor care. In the new world of CNS oncology, maximally complete resection is followed by conformal radiotherapy and often by chemotherapy, the selection of which is based on the tumor’s susceptibility to drugs that have biological actions known to interact with the tumor’s particular viii Preface molecular signature. For example, oral temozolomide is better tolerated by patients than predecessor intravenous chemotherapeutic agents and is more effective in patients with epigenetic silencing of the MGMT gene associated with DNA repair. This agent has become the standard of care for patients with anaplastic or malignant astrocytomas after resection and irradiation. In the future, we may find that today’s groundwork in immunotherapy or in stem cell biology will further improve our therapeutic reach. The chapters of this book collectively weave a tapestry that depicts the broad range of medical, surgical, and radiotherapeutic approaches to neuro-oncology as it is practiced at M. D. Anderson Cancer Center, along with the diversity of disciplines needed for effective therapy. This is now the sixth book in the M. D. Anderson Cancer Care Series. The first of these was published in 2001 and was devoted to breast cancer. This book, like the others, highlights integrated care and focuses on treating the patient through the entire spectrum of a disease. We thank Walter Pagel for shepherding the Cancer Care Series since its inception and Elizabeth Hess, Manuel Gonzales, and Tamara Locke of the Department of Scientific Publications for their dedication to producing this book. And we thank you, the reader, for your interest in this most intricate and fascinating corner of oncology in which we practice our art and our science. Franco DeMonte, MD Mark R. Gilbert, MD Anita Mahajan, MD Ian E. McCutcheon, MD C ONTENTS Foreword v Raymond Sawaya and W. K. Alfred Yung Preface vii Contributors xi Chapter 1 Epidemiology of Brain Tumors 1 Melissa L. Bondy, Randa El-Zein, and Michael E. Scheurer Chapter 2 Neuropathology and Molecular Biology of Intracranial Tumors 23 Gregory N. Fuller and Kenneth D. Aldape Chapter 3 Radiology of Brain Tumors: Structure and Physiology 37 J. Matthew Debnam, Leena Ketonen, Leena M. Hamberg, and George J.Hunter Chapter 4 Surgically Curable Brain Tumors of Adults 53 Franco DeMonte Chapter 5 Low-Grade Gliomas: Evidence-Based Treatment Options 93 Frederick F. Lang Chapter 6 Surgical Strategies for High-Grade Gliomas 121 Sujit S. Prabhu Chapter 7 Radiation Oncology for Tumors of the Central Nervous System: Improving the Therapeutic Index 135 Shiao Y. Woo x Contents Chapter 8 Cytotoxic Chemotherapy for Diffuse Gliomas 153 Ivo W. Tremont-Lukats and Mark R. Gilbert Chapter 9 Innovative Treatment Strategies for High-Grade Gliomas 171 Charles A. Conrad and Amy B. Heimberger Chapter 10 Pituitary Tumors in Oncology 191 Ian E. McCutcheon Chapter 11 Management of Lung Cancer, Breast Cancer, and Melanoma Metastatic to the Brain 225 Jeffrey S. Weinberg Chapter 12 Neoplastic Meningitis 245 Morris D. Groves Chapter 13 Lymphoma Affecting the Central Nervous System 263 Barbara Pro Chapter 14 Tumors of the Extradural Spine 273 Ehud Mendel Chapter 15 Tumors of the Spinal Cord and Intradural Space 295 Laurence D. Rhines and Morris D. Groves Chapter 16 Symptom Management for Patients with Brain Tumors: Improving Quality of Life 329 Allen W. Burton, Tracy L. Veramonti, Phillip C. Phan, and Jeffrey S. Wefel Index 353