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Unformatted text preview: Oncology of CNS Tumors Jörg-Christian Tonn David A. Reardon James T. Rutka Manfred Westphal Editors Third Edition 123 123 Oncology of CNS Tumors Jörg-Christian Tonn  •  David A. Reardon James T. Rutka • Manfred Westphal Editors Oncology of CNS Tumors Third edition 2019 Editors Jörg-Christian Tonn Department of Neurosurgery University of Munich Department of Neurosurgery Munich Germany James T. Rutka SickKids Hospital University of Toronto Toronto, ON Canada David A. Reardon Dana-Farber Cancer Institute Center Harvard University Boston USA Manfred Westphal Department of Neurosurgery University of Hamburg Hamburg Germany ISBN 978-3-030-04151-9    ISBN 978-3-030-04152-6 (eBook) Library of Congress Control Number: 2019935556 © Springer Nature Switzerland AG 2019 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland Preface Neurooncology has definitively evolved into a molecular-based medicine as many entities are now being diagnosed and classified using molecular signatures. Their clinical relevance has been validated by numerous large cohort studies. This led to a revision of the WHO Classification with major changes including the incorporation of new entities that are defined by histology and molecular features. Therefore, treatment concepts had to be revised and refined accordingly, reflecting the respective roles of microsurgery, chemoand immunotherapies, and radiation oncology. Likewise, the progress of imaging techniques within the last years needed to be considered. Thus, the third edition of this textbook had to be reorganized within the framework of the revised WHO Classification System and most chapters had to be thoroughly revised. We also added new chapters with focus on principal concepts of neurooncology and practical issues of patient care. We gratefully acknowledge the contribution of all authors who are highly renowned international experts in their field as well as Meike Stoeck and Sushil Kumar Sharma with their team from Springer-Verlag for their excellent support and assistance. Munich, Germany Boston, MA, USA Toronto, ON, Canada Hamburg, Germany Jörg-Christian Tonn David A. Reardon James T. Rutka Manfred Westphal v Contents Part I General Aspects in Neurooncology 1 Pathology and Classification of Tumors of the Central Nervous System ����������������������������������������������������������������������������������������������   3 Guido Reifenberger, Ingmar Blümcke, Pieter Wesseling, Torsten Pietsch, and Werner Paulus 2 Etiological and Epidemiological Aspects ��������������������������������������  91 Daniel I. Jacobs, E. Susan Amirian, Elizabeth B. Claus, Robert B. Jenkins, Melissa L. Bondy, and Margaret R. Wrensch 3 Imaging of Central Nervous System Tumors�������������������������������� 111 K. Ina Ly, Nathalie L. Albert, and Elizabeth R. Gerstner 4 Tumor Biology���������������������������������������������������������������������������������� 143 Farshad Nassiri, Laureen Hachem, and Gelareh Zadeh 5 Concepts of Personalized Medicine in Neuro-oncology���������������� 153 Michael Weller, Manfred Westphal, and David A. Reardon 6 Local Therapies�������������������������������������������������������������������������������� 159 Rachel Grossmann, Zvi Ram, Michael A. Vogelbaum, E. Antonio Chiocca, Manfred Westphal, Jörg-Christian Tonn, Friedrich Kreth, and Niklas Thon Part II Neurooncology of the Cranial Space 7 Tumors of the Skull Including Chordoma������������������������������������ 175 Roland Goldbrunner, Jörg-Christian Tonn, and Volker Neuschmelting 8 Meningiomas and Meningeal Tumors�������������������������������������������� 185 Manfred Westphal, Katrin Lamszus, and Jörg-Christian Tonn 9 WHO II and III Gliomas���������������������������������������������������������������� 217 Shawn L. Hervey-Jumper, M. J. van de Bent, Minesh P. Mehta, and Mitchel S. Berger 10 Glioblastoma������������������������������������������������������������������������������������ 237 Michael Weller, Colin Watts, David A. Reardon, and Minesh P. Mehta vii viii 11 Ependymomas and Tumors of the Ventricular System���������������� 249 Manfred Westphal 12 Pituitary Adenomas ������������������������������������������������������������������������ 271 Jörg Flitsch, Davis G. Taylor, and John A. Jane Jr 13 Tumors of the Pineal Region ���������������������������������������������������������� 283 Manfred Westphal 14 Tumors of the Cranial Nerves�������������������������������������������������������� 301 Jörg-Christian Tonn, Alexander Muacevic, and Roland Goldbrunner 15 Hemangioblastoma and von Hippel-Lindau Disease�������������������� 321 Ranjit Ganguly, David Dornbos III, Jonathan L. Finlay, and Russell R. Lonser 16 Orbital Tumors�������������������������������������������������������������������������������� 331 Christoph Hintschich, Ullrich Müller-Lisse, and Geoffrey E. Rose 17 Primary CNS Lymphoma��������������������������������������������������������������� 359 Lakshmi Nayak and Uwe Schlegel 18 Brain Metastases and Leptomeningeal Metastases���������������������� 377 Lynn Mubita, Ian Lee, Mira Shah, Emilie Le Rhun, and Steven Kalkanis Part III Pediatric Neurooncology 19 Neurocutaneous Syndromes������������������������������������������������������������ 389 Michael S. Taccone and James T. Rutka 20 Brainstem Tumors in Children ������������������������������������������������������ 425 Ali S. Haider, James M. Drake, and James T. Rutka 21 Supratentorial Lobar Gliomas in Childhood and Adolescence ������������������������������������������������������������������������������ 443 Cassie Kline, Anu Banerjee, and Nalin Gupta 22 Thalamic Gliomas���������������������������������������������������������������������������� 459 William B. Lo and James T. Rutka 23 Optic Pathway Gliomas������������������������������������������������������������������ 481 Ian F. Pollack 24 Ganglioglioma���������������������������������������������������������������������������������� 493 Christian Dorfer 25 Cerebellar Astrocytomas ���������������������������������������������������������������� 503 Michael C. Dewan and John C. Wellons III 26 Rare Childhood Tumors: Desmoplastic Infantile Ganglioglioma and Pleomorphic Xanthoastrocytoma������������������ 513 Gregory W. Albert Contents Contents ix 27 Ependymoma in the Children�������������������������������������������������������� 523 Shobhan Vachhrajani and Corey Raffel 28 Medulloblastoma������������������������������������������������������������������������������ 539 Claudia M. Kuzan-Fischer, Isabelle Ferry, Ana S. Guerreiro Stucklin, and Michael D. Taylor 29 Dysembryoplastic Neuroectodermal Tumors�������������������������������� 555 Aurelia Peraud, Jörg-Christian Tonn, and James T. Rutka 30 Craniopharyngioma: Current Classification, Management, and Future Directions���������������������������������������������������������������������� 561 Zohreh Habibi, Deya Abu Reesh, and James T. Rutka 31 Intracranial Germ Cell Tumors������������������������������������������������������ 585 Seung-Ki Kim, Ji Hoon Phi, Sung-Hye Park, and Kyu-Chang Wang 32 Choroid Plexus Tumors ����������������������������������������������������������������� 603 Mark M. Souweidane 33 Atypical Teratoid Rhabdoid Tumors���������������������������������������������� 615 Holly Lindsay and Annie Huang Part IV Spinal Neurooncology and Peripheral Nerve Tumors 34 Intramedullary Tumors ������������������������������������������������������������������ 633 Manfred Westphal 35 Intradural Extramedullary Tumors���������������������������������������������� 659 Roland Goldbrunner and Volker Neuschmelting 36 Epidural Tumors and Metastases �������������������������������������������������� 671 Krisztina Moldovan, Jared Fridley, Thomas Kosztowski, and Ziya Gokaslan 37 Spinal Robotic Radiosurgery���������������������������������������������������������� 695 Alexander Muacevic, Arjun Sahgal, and Jörg-Christian Tonn 38 Peripheral Nerve Sheath Tumors �������������������������������������������������� 703 Suganth Suppiah, Shirin Karimi, and Gelareh Zadeh Part V Aspects of General Care in Neurooncology 39 Epilepsy and Anticonvulsant Therapy in Brain Tumor Patients �������������������������������������������������������������������������������� 717 Sylvia C. Kurz, David Schiff, and Patrick Y. Wen 40 Hydrocephalus Related to CNS Malignancies in Adults�������������� 729 Emilie Le Rhun, Jörg-Christian Tonn, and Michael Weller 41 Gliomas and Pregnancy������������������������������������������������������������������ 737 Jacob J. Mandel, Akash Patel, and Shlomit Yust-Katz x 42 Delayed Neurologic Complications of Brain Tumor Therapy�������������������������������������������������������������������������������������������� 751 Jörg Dietrich, Sebastian F. Winter, and Michael W. Parsons 43 Quality of Life and Cognition �������������������������������������������������������� 769 Marijke B. Coomans, Linda Dirven, and Martin J. B. Taphoorn 44 Palliative Care���������������������������������������������������������������������������������� 787 C. Bausewein, S. Lorenzl, R. Voltz, M. Wasner, and G. D. Borasio Contents Part I General Aspects in Neurooncology David A. Reardon, Manfred Westphal, Jörg-­Christian Tonn 1 Pathology and Classification of Tumors of the Central Nervous System Guido Reifenberger, Ingmar Blümcke, Pieter Wesseling, Torsten Pietsch, and Werner Paulus 1.1 Introduction 1.1.1 Histologic Classification of Central Nervous System Tumors Rudolf Virchow (1821–1902), the founder of cellular pathology, already separated the gliomas from the “psammomas,” the “melanomas,” and other “sarcomas” of the nervous system in 1864/1865 [160]. However, it was not before 1926 that Bailey and Cushing developed the first systematic classification scheme for gliomas and introduced the concept of brain tumor grading [5]. The first edition of the World Health Organization (WHO) classification of tumors of the nervous system was published in 1979 [171], followed by consecutive editions in 1993, 2000, and 2007. All these WHO classifications pri- G. Reifenberger (*) Institute of Neuropathology, Heinrich Heine University, Düsseldorf, Germany e-mail: [email protected] I. Blümcke Department of Neuropathology, University Hospital Erlangen, Erlangen, Germany e-mail: [email protected] P. Wesseling Department of Pathology, VU University Medical Center, Amsterdam, The Netherlands marily relied on histologic criteria and basically followed the histogenetic principle proposed by Bailey and Cushing [5]. Based on morphologic and immunohistochemical features, each tumor entity was classified according to its presumed cell of origin. In addition to the histologic tumor typing, the WHO classification traditionally comprises a histologic grading according to a four-­ tiered scheme ranging from WHO grade I (benign) to WHO grade IV (malignant). The WHO grading is not equivalent to the histologic tumor grading commonly used in other fields of surgical pathology, but rather reflects an estimate of the presumed natural course of disease and the prognosis of the patient. In general, WHO grade I lesions include tumors with a minimal proliferative potential and the possibility of cure ­following surgical resection. Typical examples are pilocytic astrocytomas, subependymomas, myxopapillary Department of Pathology, Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands University Medical Center, Utrecht, The Netherlands e-mail: [email protected] T. Pietsch Department of Neuropathology, University of Bonn Medical Center, Bonn, Germany e-mail: [email protected] W. Paulus Institute of Neuropathology, University Hospital Münster, Münster, Germany e-mail: [email protected] © Springer Nature Switzerland AG 2019 J.-C. Tonn et al. (eds.), Oncology of CNS Tumors, 3 4 ependymomas of the cauda equina, a variety of neuronal and mixed neuronal-­ glial tumors, schwannomas, and most meningiomas. Tumors of WHO grade II are those with low mitotic activity but a tendency for recurrence. Diffuse astrocytomas, isocitrate dehydrogenase (IDH)mutant, and oligodendrogliomas, IDH-­ mutant and 1p/19q-codeleted, are classic examples of WHO grade II tumors. WHO grade III is reserved for neoplasms with histologic evidence of anaplasia, generally in the form of increased mitotic activity, increased cellularity, nuclear pleomorphism, and cellular anaplasia. WHO grade IV is assigned to mitotically active and necrosis-prone highly malignant neoplasms that are typically associated with a rapid pre- and postoperative evolution of the disease. These include the glioblastomas and the various forms of embryonal tumors [88]. 1.1.2 T  he WHO Classification 2016: Integrated Histologic and Molecular Classification Histology-based classification allowed for meaningful separation of biologically and clinically distinct brain tumor entities and thus represented the diagnostic “gold standard” for many decades. However, the histogenetic concept of central nervous system (CNS) tumor classification has been challenged as for most tumors the actual cell of origin is still unknown. Experimental evidence from mouse models suggests that gliomas, for example, are more likely to arise from neural stem or progenitor cells rather than from terminally differentiated astrocytes or oligodendrocytes [141]. More importantly, it became evident that the rapidly growing knowledge on the diagnostic and/or prognostic value of particular genetic and epigenetic alterations in different tumor types needed to be included in an integrated morphologic and molecular approach for tumor classification [90]. Several studies reported that molecular classification of, e.g., adult gliomas correlates better with clinical outcome than histologic classification. In G. Reifenberger et al. addition, it has become clear that certain tumor entities, including glioblastoma, correspond to a spectrum of genetically and biologically distinct tumor groups, whereas oligoastrocytomas lack distinctive genetic alterations but molecularly correspond to either astrocytic or oligodendroglial tumors. The revised fourth edition of the WHO classification of CNS tumors of 2016 [89, 91] has considered these advances and employs a combination of histologic and molecular characteristics for the definition of several tumor entities, in particular among the gliomas and embryonal tumors (Table 1.1). This integrated “histomolecular” approach represents a paradigm shift and allows for a more reproducible diagnosis but also may cause new challenges, e.g., in terms of the required implementation of molecular diagnostic methods [97]. Moreover, it is important to be aware of the shortcomings of molecular tests. For example, for the diagnosis of “canonical” oligodendroglioma according to the WHO classification 2016, the tumor should have complete loss of both the short arm of chromosome 1 and of the long arm of chromosome 19 (whole arm 1p/19q codeletion), but, e.g., fluorescent in situ hybridization (FISH) analysis using one probe on 1p and one on 19q does not allow to discriminate partial from complete losses [164]. In certain situations, the molecular characteristics may actually override the histologic diagnosis in the integrated diagnostic approach. For instance, in a diffuse glioma with the histologic appearance of an astrocytoma, detection of the presence of IDH1 or IDH2 hotspot mutation combined with 1p/19q codeletion leads to the diagnosis of oligodendroglioma, IDH-mutant and 1p/19q-codeleted [91]. While tumor typing has been improved by diagnostic molecular markers in the WHO classification 2016, assessment of malignancy grades is still based on traditional morphologic criteria. Table 1.2 shows an overview of WHO grades assigned to major CNS tumor entities [91]. In most instances, WHO grading continues to provide important information on a tumor’s malignancy 1  Pathology and Classification of Tumors of the Central Nervous System 5 Table 1.1  WHO classification of tumors of the central nervous system 2016 (adapted from [91]) Diffuse astrocytic and oligodendroglial tumors Diffuse astrocytoma, IDH-mutant  Gemistocytic astrocytoma, IDH-mutant Diffuse astrocytoma, IDH-wild-type Diffuse astrocytoma, NOS Anaplastic astrocytoma, IDH-mutant Anaplastic astrocytoma, IDH-wild-type Anaplastic astrocytoma, NOS Glioblastoma, IDH-wild-type  Giant cell glioblastoma  Gliosarcoma   Epithelioid glioblastoma Glioblastoma, IDH-mutant Glioblastoma, NOS Diffuse midline glioma, H3-K27M-mutant Oligodendroglioma, IDH-mutant and 1p/19q-codeleted Oligodendroglioma, NOS Anaplastic oligodendroglioma, IDH-mutant and 1p/19q-codeleted Anaplastic oligodendroglioma, NOS Oligoastrocytoma, NOS Anaplastic oligoastrocytoma, NOS Other astrocytic tumors Pilocytic astrocytoma   Pilomyxoid astrocytoma Subependymal giant cell astrocytoma Pleomorphic xanthoastrocytoma Anaplastic pleomorphic xanthoastrocytoma Ependymal tumors Subependymoma Myxopapillary ependymoma Ependymoma  Papillary ependymoma  Clear cell ependymoma  Tanycytic ependymoma Ependymoma, RELA fusion-positive Anaplastic ependymoma Other gliomas Chordoid glioma of third ventricle Angiocentric glioma Astroblastoma Choroid plexus tumors Choroid plexus papilloma Atypical choroid plexus papilloma Choroid plexus carcinoma Neuronal and mixed neuronal-glial tumors Dysembryoplastic neuroepithelial tumor Gangliocytoma Ganglioglioma Anaplastic ganglioglioma Tumors of the cranial and paraspinal nerves Schwannoma  Cellular schwannoma  Plexiform schwannoma Melanotic schwannoma Neurofibroma  Atypical neurofibroma  Plexiform neurofibroma Perineurioma Hybrid nerve sheath tumors Malignant peripheral nerve sheath tumor (MPNST)  Epithelioid MPNST  MPNST with perineurial differentiation Meningiomas Meningioma Meningothelial meningioma Fibrous meningioma Transitional meningioma Psammomatous meningioma Angiomatous meningioma Microcystic meningioma Secretory meningioma Lymphoplasmacyte-rich meningioma Metaplastic meningioma Chordoid meningioma Clear cell meningioma Atypical meningioma Papillary meningioma Rhabdoid meningioma Anaplastic (malignant) meningioma Mesenchymal, non-meningothelial tumors Solitary fibrous tumor/hemangiopericytoma Hemangioblastoma Hemangioma Epithelioid hemangioendothelioma Angiosarcoma Kaposi sarcoma Ewing sarcoma/peripheral primitive neuroectodermal tumor Lipoma Angiolipoma Liposarcoma Desmoid-type fibromatosis Myofibroblastoma Inflammatory myofibroblastic tumor Benign fibrous histiocytoma Fibrosarcoma Undiffer...
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