Neuro-Oncology Part I

Wolfgang Grisold, Riccardo Soffietti

• Publisher: Elsevier
• Year: 2012
• Print ISBN:
  9780444521385
• eBook ISBN:
  9780444534958
• Topic: Medicine
• Subtopic: Neurology

Handbook of Clinical Neurology: Neuro-Oncology, Part I summarizes the present state of scientific and clinical knowledge in the field of neuro-oncology, including information related to diagnostic techniques such as imaging, along with immunology, molecular biology, and clinical aspects of tumors. Management and new therapeutic strategies for tumors, including gene therapy and molecularly targeted treatments, are also covered. Divided into eight sections encompassing 61 chapters, the book begins with an overview of the basic principles of tumors, including the epidemiology of primary central nervous system tumors, angiogenesis and invasion in cancer, the link between blood-brain barrier and brain edema, and the role of stem cells in gliomas. It proceeds with a discussion of diagnostic tools such as neuroimaging, the principles of tumor therapy such as radiotherapy and immunotherapy, and clinical trials in neuro-oncology. The reader is also introduced to specific tumor types such as low-grade gliomas, anaplastic astrocytomas, and medulloblastoma and primitive neuroectodermal tumors, along with rare brain tumors like neurofibromatosis and other genetic syndromes. Furthermore, the book explains the neurological complications of systemic cancer and complications from treatments. This volume will appeal to clinicians and neuroscientists as well as researchers who want to gain a better understanding of the clinical features and management of the neurological manifestations of tumors. - An invaluable resource that includes critical, in-depth insights into recent developments in neuro-oncology - A fresh perspective on molecular biology, immunology, and other clinical aspects of tumors of the nervous system - Extensive coverage of tumor management and new therapeutic strategies, including gene therapy and molecularly targeted treatments - New tactics and therapies that will aid clinicians in their quest to provide optimal care for their neuro-oncological patients

Handbook of Clinical Neurology, Vol. 104, No. Suppl C, 2012

ISSN: 0072-9752

doi: 10.1016/B978-0-444-52138-5.00001-3

Chapter 1Epidemiology of primary central nervous system tumors

Graziella Filippini*

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Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

^*Correspondence to: Graziella Filippini, Istituto Nazionale Neurologico ‘C. Besta’, Via Celoria 11, 20133 Milan, Italy.

E-mail address: [email protected]

Abstract

The descriptive epidemiology of primary central nervous system (CNS) tumors is based on population-based cancer registries that include tumors of the brain, cranial nerves, cerebral meninges, spinal cord, and spinal meninges. Malignant CNS tumors in adults account for 1.7% of new cancers and 2.1% of cancer deaths. In Europe, Australia/New Zealand, and North America there are 7.5–14 new cases per 100 000 population per year in males, and 4–11 new cases in females. Incidence rates of benign and borderline CNS tumors are available from the Surveillance, Epidemiology, and End Results (SEER) Program for the time period 1975–2004: incidence was 8.1 new cases per 100 000 population per year in males, and 12.1 in females. Incidence and mortality significantly increased in white males and females from 1975–1991; subsequently, the incidence remained steady, and mortality decreased significantly from 1992–2004. In population-based studies less than 5% of glioma risk is hereditary. Well-identified genetic syndromes that include primary brain tumors (PBTs) are most often autosomal dominant, and have variable penetrance. The most common syndrome is neurofibromatosis type 1. Ionizing radiation has a proven etiological role in experimental studies in monkeys and primates and, as a late complication of therapeutic X-irradiation, in humans. Extensive epidemiological research conducted during the past 20 years on occupational electromagnetic field exposure did not indicate strong or consistent associations with adult PBTs. The WHO/IARC has classified RF electromagnetic fields as possibly carcinogenic to humans (Group 2B) based on an increased risk for glioma associated with wireless phone use. Additional research needs to be conducted into the long-term use of mobile phones.

Introduction

The descriptive epidemiology of the central nervous system (CNS) tumors is based on population-based cancer registries that include tumors of the brain, cranial nerves, cerebral meninges, spinal cord, and spinal meninges.

The anatomical classification of tumors used in cancer registries has followed the evolution of the World Health Organization (WHO) International Classification of Diseases (ICD), now in its 10th revision (ICD-10; WHO, 1992), and the coding scheme for oncology, the International Classification of Diseases for Oncology (ICD-O, 3rd edition; WHO, 2000). CNS tumors are represented by the ICD-O topographical codes: C70.0–9, meninges; C71.0–9, brain; C72.0–9, spinal cord, cranial nerves, and other parts of the CNS; C75.1–C75.3, the pituitary and pineal gland. Histological subgroups are defined by the morphological terms of the ICD-O and behavior is coded /0 for benign tumors, /1 for low or uncertain malignant potential or borderline malignancy, /2 for in situ lesions and /3 for malignant tumors (Table 1.1). In population-based cancer registries the topographical codes C70–72 are grouped together in the ‘brain, nervous system’ group, and only tumors of malignant behavior (code /3) are included and combined.

Table 1.1 World Health Organization classification of primary central nervous system tumors (ICD-O, 2000)

Histological type	ICD-O morphology code/behavior code*
Tumors of neuroepithelial tissue Astrocytic tumors
Diffuse astrocytoma	9400/3
Fibrillary astrocytoma	9420/3
Protoplasmic astrocytoma	9410/3
Gemistocytic astrocytoma	9411/3
Anaplastic astrocytoma	9401/3
Glioblastoma	9440/3
Giant cell glioblastoma	9441/3
Gliosarcoma	9442/3
Pilocytic astrocytoma	9421/1
Pleomorphic xanthoastrocytoma	9424/3
Subependymal giant cell astrocytoma	9384/1
Oligodendroglial tumors
Oligodendroglioma	9450/3
Anaplastic oligodendroglioma	9451/3
Mixed gliomas
Oligoastrocytoma	9382/3
Ependymal tumors
Ependymoma	9391/3
Anaplastic ependymoma	9392/3
Myxopapillary ependymoma	9394/1
Subependymoma	9383/1
Choroid plexus tumors
Choroid plexus papilloma	9390/0
Choroid plexus carcinoma	9390/3
Glioma, NOS	9380
Glial tumors of uncertain origin
Astroblastoma	9430/3
Gliomatosis cerebri	9381/3
Neuronal and mixed neuronal–glial tumors
Gangliocytoma	9492/0
Ganglioglioma	9505/1
Pineal parenchymal tumors
Pineocytoma	9361/1
Pineoblastoma	9362/3
Embryonal tumors
Medulloepithelioma	9501/3
Ependymoblastoma	9392/3
Medulloblastoma	9470/3
Supratentorial primitive neuroectodermal tumor (PNET)	9473/3
Neuroblastoma	9500/3
Meningioma
Meningioma, NOS	9530
Meningotheliomatous meningioma	9531
Fibrous meningioma	9532
Psammomatous meningioma	9533
Hemangioblastic meningioma	9535
Hemangiopericytic meningioma	9536
Transitional (mixed) meningioma	9537

NOS, not otherwise specified.

* Morphology code of the International Classification of Diseases for Oncology (ICD-O). Behavior is coded /0 for benign tumors, /1 for low or uncertain malignant potential or borderline malignancy, (/2 for in situ lesions), and /3 for malignant tumors.

Adapted and reprinted with permission from WHO (2000). ©WHO.

Since the publication of the first edition of the ICD-O (WHO, 1976), cancer registries have adapted their operative systems to the standard classification and coding rules, but the definition, classification, and grouping of cancers have varied considerably among registries. Therefore, caution is required when comparing incidence rates over time, because the observed differences may be the result of changes in the methodology used by the different registries.

Descriptive epidemiology of adult malignant and nonmalignant central nervous system tumors

Incidence and mortality

Comprehensive population-based estimates of incidence and mortality from malignant CNS tumors are available from different sources. GLOBOCAN 2002 was a project of the International Agency for Research on Cancer (IARC) that used incidence and mortality data from cancer registries worldwide between 1993 and 2001 (Ferlay et al., 2004) (Figure 1.1). The registries included in the GLOBOCAN covered entire national populations or samples of such populations from selected regions. The 9th volume of Cancer Incidence in Five Continents provided incidence and mortality data from populations all over the world for the time period 1998–2002 (Curado et al., 2007). The Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute has published incidence and mortality data for the period 1975–2004 from population-based cancer registries covering approximately 26% of the US population (Ries et al., 2007). Based on the statistics from these programs, malignant CNS tumors account for 1.7% of new cancers and 2.1% of cancer deaths. The highest rates are observed in more developed areas. In Europe, Australia/New Zealand, and North America there are 7.5–14 new cases per 100 000 population per year in males, and ...

Table of contents

• Cover
• Table of Contents
• Series Page
• Copyright
• Handbook of Clinical Neurology 3rd Series
• Foreword
• Preface
• List of contributors
• Chapter 1: Epidemiology of primary central nervous system tumors
• Chapter 2: Molecular biology of brain tumors
• Chapter 3: Angiogenesis and invasion in cancer
• Chapter 4: Immunology of brain tumors
• Chapter 5: Blood–brain barrier and brain edema
• Chapter 6: Stem cells in gliomas
• Chapter 7: Diagnostic neuropathology of tumors of the central nervous system
• Chapter 8: Neuroimaging
• Chapter 9: Functional imaging in brain surgery
• Chapter 10: Imaging brain tumors with PET, SPECT, and ultrasonography
• Chapter 11: CSF and laboratory analysis (tumor markers)
• Chapter 12: Electrophysiology and intraoperative neurophysiological monitoring
• Chapter 13: Evaluation and monitoring of peripheral nerve function
• Chapter 14: Evaluation of cognitive functions and quality of life
• Chapter 15: Principles of brain tumor surgery
• Chapter 16: Radiotherapy
• Chapter 17: Chemotherapy with cytotoxic and cytostatic agents in brain cancer
• Chapter 18: Molecularly targeted therapy in neuro-oncology
• Chapter 19: Inhibiting angiogenesis in malignant gliomas
• Chapter 20: Immunotherapy of brain tumors
• Chapter 21: Gene therapy
• Chapter 22: Chemotherapy for brain tumors with polymer drug delivery
• Chapter 23: Convection-enhanced delivery of therapeutic agents into the brain
• Chapter 24: Management of cognitive deficits and mood disturbance
• Chapter 25: Steroids and brain tumors
• Chapter 26: Seizure control in brain tumors
• Chapter 27: Cancer pain management and palliative care
• Chapter 28: Clinical trials in neuro-oncology
• Index