You've reached the end of your free preview.
Want to read all 777 pages?
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...
View
Full Document
- Fall '19
- Oncology, Brain tumor, Astrocytoma, Glioblastoma