Russell and Rubinstein’s Pathology of Tumors of the Nervous System This page intentionally left blank Russell and Rubinstein’s Pathology of Tumors of the Nervous System Seventh edition Roger E. McLendon MD Professor of Pathology, Director of Anatomic Pathology Services, Chief, Sections of Surgical Pathology and Neuropathology, Dept of Pathology, Duke University Medical Center, Durham, North Carolina, USA Marc K. Rosenblum MD Chairman of Department of Pathology, Chief of Neuropathology and Autopsy Service, James Ewing Alumni Chair of Pathology, Memorial Sloan-Kettering Cancer Center, New York, USA Darell D. Bigner MD Edwin L Jones Jr & Lucille Jones Cancer Research Professor of Pathology (Neuropathology), Vice Chairman for Investigative Pathology, and Director, Pediatric Brain Tumor Foundation Institute at Duke, Director, Preston Robert Tisch Brain Tumor Center at Duke, Duke University Medical Center, Durham, North Carolina, USA Hodder Arnold AMEMBER OF THE HODDER HEADLINE GROUP This seventh edition published in 2006 by Hodder Arnold, an imprint of Hodder Education and a member of the Hodder Headline Group, 338 Euston Road, London NW1 3BH http://www.hoddereducation.com Distributed in the United States of America by Oxford University Press Inc., 198 Madison Avenue, New York, NY10016 Oxford is a registered trademark of Oxford University Press Copyright in the Seventh Edition ©2006 Roger E. McLendon, Marc K. Rosenblum, and Darell D. Bigner exclusive of material reused from the Fifth Edition ©1989 Mary M. Herman and of material reused from the Sixth Edition ©1998, Darell D. Bigner, and of the figures from Chapter 56, Primary tumors of cranial, spinal and peripheral nerves © 2006 James M. Woodruff. All rights reserved. Apart from any use permitted under UK copyright law, this publication may only be reproduced, stored or transmitted, in any form, or by any means with prior permission in writing of the publishers or in the case of reprographic production in accordance with the terms of licences issued by the Copyright Licensing Agency. In the United Kingdom such licences are issued by the Copyright Licensing Agency: 90 Tottenham Court Road, London W1T 4LP Whilst the advice and information in this book are believed to be true and accurate at the date of going to press, neither the author[s] nor the publisher can accept any legal responsibility or liability for any errors or omissions that may be made. In particular (but without limiting the generality of the preceding disclaimer) every effort has been made to check drug dosages; however it is still possible that errors have been missed. Furthermore, dosage schedules are constantly being revised and new side-effects recognized. For these reasons the reader is strongly urged to consult the drug companies’ printed instructions before administering any of the drugs recommended in this book. British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Cataloging-in-Publication Data A catalog record for this book is available from the Library of Congress ISBN-10 [normal] 0 340 810 076 ISBN-13 978 0 340 810 076 1 2 3 4 5 6 7 8 9 10 Commissioning Editor: Philip Shaw Project Editor: Heather Fyfe Production Controller: Karen Tate Cover Designer: Tim Pattinson Typeset in 10/12 pts Minion by Charon Tec Ltd, Chennai, India www.charontec.com Printed and bound in Italy What do you think about this book? Or any other Hodder Arnold title? Please send your comments to [email protected] This eBook does not include the ancillary media that was packaged with the printed version of the book. “This edition is dedicated to the memories and lives’ work of Dorothy S. Russell and Lucien J. Rubinstein.” Roger K. McLendon Marc K. Rosenblum Darell D. Bigner May 1, 2006 Dorothy S Russell, 1895–1983 Lucien J Rubinstein, 1924–1990 This page intentionally left blank Contents Contributors xi Preface xvii Acknowledgements xix List of abbreviations used xxi 1 Classification of tumors of the nervous system: an historical annotation 1 Roger E. McLendon and Paul Kleihues 2 Epidemiology of brain tumors: incidence, survival, and risk factors 11 Faith G. Davis and Bridget McCarthy 3 Nervous system tumors and atomic bomb experience of Hiroshima and Nagasaki 51 Kazunori Arita, Kaoru Kurisu and Kazuhiko Sugiyama 4 Diffuse astrocytoma 57 Roger E. McLendon, Linda Gray, Lubdha Shah, and Allan H. Friedman 5 Anaplastic astrocytoma 75 Roger E. McLendon, James M. Provenzale, and Allan H. Friedman 6 Astrocytoma of the brainstem 89 Roger E. McLendon, James M. Provenzale, and Allan H. Friedman 7 Astrocytoma of the spinal cord 97 Roger E. McLendon, James M. Provenzale, and Allan H. Friedman 8 Glioblastoma 103 Roger E. McLendon, Allan H. Friedman, and Linda Gray 9 Pilocytic astrocytoma and pilomyxoid astrocytoma 131 Thomas J. Cummings 10 Pleomorphic xanthoastrocytoma 147 Roger E. McLendon, Linda Gray, Lubdha M. Shah, and Allan H. Friedman 11 Astroblastoma 157 Marc K. Rosenblum 12 Oligodendroglioma 167 Roger E. McLendon, Johan M. Kros, Janet Bruner, and Linda Gray 13 Anaplastic oligodendroglioma and mixed glioma 187 Roger E. McLendon, Johan M. Kros, Linda Gray, and Janet Bruner 14 Ependymoma, subependymoma, and myxopapillary ependymoma 201 Ho Keung Ng 15 Monomorphous angiocentric glioma/angiocentric neuroepithelial tumor 223 Marc K. Rosenblum vii viii Contents 16 Chordoid glioma of the third ventricle 229 Daniel J. Brat 17 Choroid plexus tumor 235 Ho Keung Ng and Werner Paulus 18 Medulloblastoma 247 David W. Ellison, Steven C. Clifford, and Felice Giangaspero 19 Cerebellar liponeurocytoma 265 Giovanna Cenacchi and Felice Giangaspero 20 Atypical teratoid/rhabdoid tumor 271 Marco Gessi and Felice Giangaspero 21 Melanotic neuroectodermal tumor of infancy 277 Edward Bossen 22 Olfactory neuroblastoma 281 Edward Bossen 23 Medulloepithelioma 287 Felice Giangaspero 24 Supratentorial primitive neuroectodermal tumor: neuroblastoma, ganglioneuroblastoma, malignant neuroblastic-glial tumor and ependymoblastoma 291 Roger E. McLendon and Linda Gray 25 Ganglion cell tumors: gangliocytoma, ganglioglioma and anaplastic ganglioglioma 305 Richard A. Prayson 26 Desmoplastic infantile astrocytoma/desmoplastic infantile ganglioglioma 321 Marc K. Rosenblum 27 Extraventricular neurocytomas and unconventional glioneuronal tumors 333 Marc K. Rosenblum 28 Central neurocytoma 351 Marc K. Rosenblum 29 Dysembryoplastic neuroepithelial tumor 367 Marc K. Rosenblum 30 Dysplastic gangliocytoma of the cerebellum (Lhermitte–Duclos disease) 379 Marc K. Rosenblum and Anthony T. Yachnis 31 Hypothalamic neuronal hamartoma 391 Marc K. Rosenblum 32 Paragangliomaof the spine and skull base 401 Hernando Mena 33 Tumors of pineal parenchymal and glial cells 413 Anne Jouvet, François Fauchon, Eric Bouffet, Ghislaine Saint-Pierre, Jacques Champier, and Michelle Fèvre Montange 34 Meningiomas 427 Arie Perry 35 Solitary fibrous tumors 475 Arie Perry 36 Primary meningeal sarcoma 479 Arie Perry 37 Hemangiopericytoma 483 Edward Bossen Contents ix 38 Capillary hemangioblastoma 489 Anthony T. Yachnis 39 Cerebral sarcoma 509 B. K. Kleinschmidt-DeMasters 40 Rhabdomyosarcoma and leiomyosarcoma 521 B. K. Kleinschmidt-DeMasters 41 Angiosarcoma and Kaposi’s sarcoma 529 B. K. Kleinschmidt-DeMasters 42 Hemangioendothelioma 535 B. K. Kleinschmidt-DeMasters 43 Meningeal melanocytoma, neurocutaneous melanosis and primary meningeal melanoma 541 Daniel J. Brat 44 Germ cell tumors 551 Marc K. Rosenblum 45 Totipotential cells of the distal spinal cord 579 Thomas J. Cummings and Timothy M. George 46 Dermoid cyst, epidermoid cyst, and dermal sinus 583 Gregory N. Fuller and Teresa Ribalta 47 Intracranial cysts of endodermal or respiratory epithelial origin 591 Hidehiro Takei, Gregory N. Fuller, and Suzanne Z. Powell 48 Lipomas and rare lipomatous tumors 599 B. K. Kleinschmidt-DeMasters 49 Arachnoid cyst 607 Teresa Ribalta and Gregory N. Fuller 50 Neuroepithelial cysts of the IV ventricle and extraventricular ependymal cysts 611 Teresa Ribalta and Gregory N. Fuller 51 Ectopias and hamartomas 613 Roger E. McLendon and Thomas J. Cummings 52 Tumors of the retina 623 Thomas J. Cummings and Gordon K. Klintworth 53 Gliomas of the optic nerve and chiasm 651 Thomas J. Cummings and Gordon K. Klintworth 54 Neoplasms of the sellar region 663 M. Beatriz S. Lopes, Kamal Thapar, Eva Horvath and Kalman Kovacs 55 Chordoma and related lesions, chondrosarcoma and osteosarcoma of the cranium 765 Andrew E. Rosenberg 56 Primary tumors of cranial, spinal and peripheral nerves 787 Cristina R. Antonescu and James M. Woodruff 57 Nervous system involvement by lymphoma, leukemia, and other hematopoietic cell proliferations 837 Susan Morgello and Anand S. Lagoo 58 Neurofibromatosis type 1 903 David H. Gutmann and Arie Perry 59 Neurofibromatosis type 2 917 Anat Stemmer-Rachamimov, Fabio Nunes, and James Gusella