Brain Tumors

1 Cytogenesis of the Central Nervous System.- 1.1 Neurogenesis and Gliogenesis.- 1.2 Gliogenesis in Adult Animals.- 1.3 Development of the Cerebellar Cortex.- 1.4 Radial Glia and Ependyma.- 1.5 Genes Controlling Nervous System Development.- 2 Factors of the Transformation Process.- 2.1 Genetics and Molecular Biology.- 2.2 Familial Incidence of Tumors.- 2.3 Congenital Tumors.- 2.4 Risk Factors: Epidemiological Data.- 2.4.1 Family Characteristics.- 2.4.2 Individual Factors.- 2.4.3 Multiple Sclerosis.- 2.4.4 Virus.- 2.4.5 Head Injuries.- 2.4.6 Irradiation.- 2.4.7 Nonprofessional Exogenous Exposures.- 2.4.8 Professional Exogenous Exposures.- 2.4.9 Multiple Tumors.- 3 Experimental Tumors.- 3.1 Chemical Carcinogenesis.- 3.1.1 Topically Acting Carcinogens.- 3.1.2 Resorptive Carcinogens.- 3.1.2.1 Pathogenesis of Nitrosourea-Induced Tumors.- 3.1.2.2 Cellular Composition.- 3.1.2.3 Vascularization of ENU-Induced Tumors.- 3.1.2.4 Utilization of MNU-ENU Models.- 3.2 Viral Carcinogenesis.- 3.3 Transplantable Animal Models.- 3.4 Gene Transfer Models of Neural Tumors.- 4 Antigens of Phenotypic Expression and Differentiation Markers.- 4.1 Brain Tumor-Associated Antigens.- 4.2 Antigens Employed in the Histological Diagnosis of Brain Tumors.- 4.2.1 Glial Markers.- 4.2.1.1 S-100 Protein.- 4.2.1.2 Glial Fibrillary Acidic Protein.- 4.2.1.3 Glutamine Synthetase.- 4.2.1.4 Carbonic Anhydrase.- 4.2.1.5 Myelin Basic Protein.- 4.2.1.6 Myelin-Associated Glycoprotein.- 4.2.2 Neuronal Markers.- 4.2.2.1 Neuronal-Specific Enolase.- 4.2.2.2 Neurofilaments.- 4.2.2.3 Synaptophysin and Chromogranin.- 4.2.3 Markers Nonspecific for the Nervous System.- 4.2.4 Vessel Markers.- 4.2.5 Other Intermediate Filaments.- 4.2.6 Epithelial Membrane Antigens.- 4.2.7 Markers for Cerebral Metastases.- 5 Pathology of the Host-Tumor Interaction.- 5.1 Peritumoral Changes.- 5.1.1 Glial Reaction.- 5.1.2 Included Neurons.- 5.1.3 Ventricular Walls.- 5.2 Regressive Events in the Tumor.- 5.3 Cerebral Edema.- 5.3.1 Definition and Pathogenesis.- 5.3.2 Morphological Changes and Sequelae.- 5.4 Calcifications.- 5.5 Immune Response.- 6 Classification and Nosography of Neuroepithelial Tumors.- 7 The Concept of Malignancy: Anaplasia, Cell Proliferation, Metastasis.- 7.1 General Considerations.- 7.2 Cell Kinetics.- 7.3 Metastasis.- 7.4 Expansion and Invasiveness.- 7.4.1 Invasion Mechanisms.- 8 Descriptive Epidemiology of Primary Nervous System Tumors.- 8.1 General Data.- 8.1.1 Mortality.- 8.1.2 Incidence.- 8.2 Epidemiology of Intracranial Tumors.- 8.2.1 Histological Type.- 8.2.2 Age.- 8.2.3 Sex.- 8.2.4 Race.- 8.3 Epidemiology of Intraspinal Tumors.- 9 Astrocytic Tumors.- 9.1 Nosological Problems.- 9.2 Astrocytic Tumors of the Cerebral Hemispheres.- 9.2.1 Astrocytomas.- 9.2.1.1 Frequency, Age, Site and Clinical Features.- 9.2.1.2 Macroscopic Appearance and Imaging.- 9.2.1.3 Microscopic Appearance.- 9.2.1.4 Pilocytic Astrocytoma.- 9.2.1.5 Anaplastic Variant.- 9.2.1.6 Prognosis and Treatment of Hemispheric Astrocytomas.- 9.2.2 Glioblastoma Multiforme.- 9.2.2.1 General Considerations.- 9.2.2.2 Frequency, Age, Site and Clinical Features.- 9.2.2.3 Macroscopic Appearance and Imaging.- 9.2.2.4 Microscopic Appearance.- 9.2.2.5 Tumor Spreading.- 9.2.2.6 Differential Diagnosis.- 9.2.2.7 Giant Cell Variant.- 9.2.2.8 Gliosarcoma.- 9.2.2.9 Blood Vessel Architecture and Angiogenesis in Gliomas.- 9.2.2.10 Cellular Kinetics.- 9.2.2.11 Metabolism.- 9.2.2.12 Prognosis and Treatment.- 9.2.3 Gliomatosis Cerebri.- 9.2.4 Pleomorphic Xanthoastrocytoma.- 9.2.4.1 Macroscopic Appearance.- 9.2.4.2 Microscopic Appearance.- 9.2.4.3 Prognosis.- 9.2.5 Subependymal Giant Cell Astrocytoma (Tuberous Sclerosis).- 9.2.6 Astroblastoma.- 9.3 Astrocytic Tumors of the Midline.- 9.3.1 Astrocytoma of the Optic Nerve.- 9.3.2 Astrocytoma of the Chiasm.- 9.3.3 Brain Stem Astrocytomas.- 9.3.4 Other Midline Astrocytomas.- 9.3.5 Cerebellar Astrocytomas.- 9.3.5.1 Nosographic Considerations.- 9.3.5.2 Frequency, Age.- 9.3.5.3 Macroscopic Appearance.- 9.3.5.4 Microscopic Appearance.- 9.3.5.5 Rosenthal’s Fibers.- 9.3.5.6 Malignant Transformation, Prognosis.- 9.4 Astrocytic Tumors of the Spinal Cord.- 9.4.1 Frequency, Age.- 9.4.2 Macroscopic and Microscopic Appearance.- 10 Oligodendroglial Tumors.- 10.1 Oligodendroglioma.- 10.1.1 Frequency, Age, Site and Clinical Features.- 10.1.2 Macroscopic Appearance and Imaging.- 10.1.3 Microscopic Appearance.- 10.2 Presence of Astrocytes and the Problem of Mixed Gliomas: Oiigoastrocytoma.- 10.3 Anaplastic Oligodendroglioma and Prognosis.- 11 Ependymal Tumors.- 11.1 Ependymoma.- 11.1.1 Classification Problems.- 11.1.2 Frequency, Age, Sex, Site and Clinical Features.- 11.1.3 Macroscopic Appearance and Imaging.- 11.1.4 Microscopic Appearance.- 11.1.5 Regressive Events.- 11.1.6 Immunohistochemistry.- 11.1.7 Electron Microscopy.- 11.1.8 Anaplastic Ependymoma.- 11.1.9 Spread Via the Cerebrospinal Fluid.- 11.1.10 Treatment and Prognosis.- 11.2 Subependymoma.- 11.3 Ependymoblastoma.- 12 Choroid Plexus Tumors.- 12.1 Plexus-Papilloma.- 12.1.1 Frequency, Age, Site and Clinical Features.- 12.1.2 Macroscopic Appearance and Imaging.- 12.1.3 Microscopic Appearance.- 12.1.4 Treatment and Prognosis.- 12.2 Malignant Variant (Plexus Carcinoma).- 13 Tumors Composed of Neural Cells.- 13.1 Ganglioglioma (Gangliocytoma).- 13.1.1 Frequency, Age, Site and Clinical Features.- 13.1.2 Macroscopic Appearance and Imaging.- 13.1.3 Microscopic Appearance.- 13.1.4 Malignant Transformation (Malignant Ganglioglioma).- 13.1.5 Prognosis.- 13.2 Dysplastic Gangliocytoma of the Cerebellum.- 13.3 Infantile Desmoplastic Ganglioglioma -Desmoplastic Infantile Astrocytoma.- 13.4 Central Neurocytoma.- 13.5 Dysembryoplastic Neuroepithelial Tumors.- 13.6 Olfactory Neuroblastoma.- 14 Pineal Gland Tumors.- 14.1 The Pineal Gland.- 14.2 Pineal Gland Tumors.- 14.2.1 Pineocytoma.- 14.2.1.1 Macroscopic Appearance.- 14.2.1.2 Microscopic Appearance.- 14.2.1.3 Treatment and Prognosis.- 14.2.2 Pinealoblastoma.- 14.2.2.1 Macroscopic Appearance.- 14.2.2.2 Microscopic Appearance.- 14.2.2.3 Prognosis.- 14.2.4 Trilateral Retinoblastoma.- 14.3 Pineal Cysts.- 15 Embryonal Tumors.- 15.1 Medulloepithelioma.- 15.2 Medulloblastoma.- 15.2.1 Frequency, Age and Clinical Features.- 15.2.2 Macroscopic Appearance and Imaging.- 15.2.3 Microscopic Appearance.- 15.2.3.1 Desmoplastic Variant.- 15.2.3.2 Melanotic Medulloblastoma.- 15.2.3.3 Medullomyoblastoma.- 15.2.4 DNA Content and Pathology.- 15.2.5 Problem of Differentiation.- 15.2.6 Prognosis, Recurrence, Metastasis.- 15.2.7 Medulloblastoma of Adults.- 15.3 Neuroblastoma.- 15.3.1 Macroscopic Appearance.- 15.3.2 Microscopic Appearance.- 15.3.3 Prognosis.- 15.4 Polar Spongioblastoma.- 15.5 Appendix: Tumors of the Retina.- 15.5.1 Retinoblastoma.- 16 Glomus Tumors, Paragangliomas.- 16.1 Site, Age, and Clinical Features.- 16.2 Macroscopic Appearance and Imaging.- 16.3 Microscopic Appearance.- 16.4 Prognosis.- 17 Tumors of the Cranial and Spinal Nerves.- 17.1 Neurinoma (Schwannoma).- 17.1.1 Frequency, Age, Sex.- 17.1.2 Site.- 17.1.3 Clinical Features.- 17.1.4 Macroscopic Appearance and Imaging.- 17.1.5 Microscopic Appearance.- 17.1.6 Cellular Schwannoma.- 17.1.7 In Vitro Culture.- 17.2 Neurofibromas.- 17.2.1 Plexiform Neurofibromas.- 17.3 Granular Cell Tumors.- 17.4 Neurothekeoma.- 17.5 Perineurioma.- 17.6 Prognosis, Malignancy.- 18 Tumors of the Meninges.- 18.1 Meningiomas.- 18.1.1 General Considerations and Nomenclature.- 18.1.2 Frequency.- 18.1.3 Age.- 18.1.4 Sex.- 18.1.5 Familial Tendency.- 18.1.6 Trauma and Irradiation.- 18.1.7 Association with Other Tumors.- 18.1.8 Site.- 18.1.9 Multiple Meningiomas.- 18.1.10 Clinical Features and Imaging.- 18.1.11 Macroscopic Appearance.- 18.1.12 Microscopic Appearance.- 18.1.12.1 Angiomatous Meningiomas.- 18.1.12.2 Malignant Meningioma.- 18.1.13 Metaplasia in Meningiomas.- 18.1.14 Regressive Changes.- 18.1.15 Calcifications.- 18.1.16 Electron Microscopy.- 18.1.17 Receptors for Steroid Hormones.- 18.1.18 In Vitro Culture.- 18.1.19 Growth Modality.- 18.1.20 Metastasis.- 18.1.21 Prognosis, Treatment.- 18.2 Other Mesenchymal Tumors of the Meninges.- 18.2.1 Benign Neoplasms.- 18.2.2 Malignant Neoplasms.- 18.2.2.1 Hemangiopericytoma.- 18.2.2.2 Fibrosarcoma.- 18.2.2.3 Malignant Fibrous Histiocytoma.- 18.2.2.4 Primary Meningeal Sarcomatosis.- 18.2.2.5 Primitive Melanoblastosis of the Leptomeninges.- 18.2.2.6 Primary Melanotic Lesions.- 18.2.2.7 Meningiomatosis or Meningoangiomatosis.- 18.2.2.8 Miscellaneous.- 19 Mesenchymal Tumors.- 19.1 Chordomas.- 19.1.1 General Considerations.- 19.1.2 Macroscopic Appearance.- 19.1.3 Microscopic Appearance.- 19.1.4 Electron Microscopy.- 19.1.5 Differential Diagnosis.- 19.1.6 Prognosis.- 19.2 Chondroma.- 19.3 Chondrosarcomas.- 19.4 Osteomas.- 19.5 Osteosarcoma.- 20 Vascular Tumors.- 20.1 Capillary Hemangioblastoma.- 20.1.1 Biological Data.- 20.1.2 Macroscopic Appearance.- 20.1.3 Microscopic Appearance.- 20.1.4 Regressive Events.- 20.1.5 Electron Microscopy Study.- 20.1.6 Metastasis, Recurrences, Prognosis.- 20.1.7 Associated Polycythemia.- 20.1.8 Differential Diagnosis.- 21 Tumors and Dysontogenetic Lesions.- 21.1 Germ Cell Tumors.- 21.1.1 Frequency, Age, Sites, Clinical Features, Imaging.- 21.1.2 Pathogenesis.- 21.1.3 Germinoma.- 21.1.3.1 Macroscopic Appearance.- 21.1.3.2 Microscopic Appearance.- 21.1.3.3 Prognosis, Treatment.- 21.1.4 Embryonal Carcinoma.- 21.1.5 Choriocarcinoma.- 21.1.6 Endodermal Sinus Tumor.- 21.1.7 Teratocarcinoma.- 21.1.8 Immunohistochemical and Chemical Characterization.- 21.2 Teratomas.- 21.2.1 Frequency, Age, Site.- 21.2.2 Macroscopic Appearance.- 21.2.3 Microscopic Appearance.- 21.2.4 Prognosis, Recurrence.- 21.3 Tumors with Muscle Cells.- 21.3.1 Medullomyoblastoma.- 21.3.2 Primitive CNS Rhabdomyosarcoma.- 21.3.3 Other Tumors.- 21.3.4 Rhabdoid Tumors.- 21.4 Dermo-epidermoid Cysts.- 21.4.1 Nosography, Pathogenesis.- 21.4.2 Frequency, Age, Site and Clinical Features.- 21.4.3 Macroscopic Appearance and Imaging.- 21.4.4 Microscopic Appearance.- 21.4.5 Prognosis, Sequelae.- 21.5 Craniopharyngioma and Epithelial Cysts.- 21.5.1 Embryogenetic Aspects.- 21.5.2 Incidence.- 21.5.3 Site.- 21.5.3.1 Intraventricular Tumors.- 21.5.4 Clinical Aspects.- 21.5.5 Macroscopic Appearance.- 21.5.6 Microscopic Appearance.- 21.5.6.1 Electron Microscopy and Immunohistochemistry.- 21.5.6.2 Calcification.- 21.5.6.3 Cystic Component.- 21.5.7 Adjacent Tissue.- 21.5.8 Relationships of Craniopharyngiomas with Rathke’s Fissure Cysts.- 21.5.9 Prognosis, Treatment.- 21.6 Neuroepithelial and Non-Neuroepithelial Cysts.- 21.6.1 Colloid Cysts of the Third Ventricle.- 21.6.1.1 Frequency, Age, Site.- 21.6.1.2 Macroscopic Appearance.- 21.6.1.3 Microscopic Appearance.- 21.6.2 Spinal Enterogenous Cysts.- 21.6.3 Arachnoid Cysts.- 21.7 Lipomas.- 21.7.1 Frequency, Age, Site.- 21.7.2 Macroscopic Appearance.- 21.7.3 Microscopic Appearance.- 21.7.4 Prognosis, Treatment.- 21.8 Hamartomas, Ectopias, and Ectopic Tumors.- 21.8.1 Hamartoma of the Hypothalamus.- 21.8.2 Granule Cell Tumors.- 21.8.3 Meningeal Gliomas.- 21.8.4 Ec topic Gliomas and Neural Hamartomas.- 21.9 Hamartomas or Vascular Malformations.- 21.9.1 Clinical Features.- 21.9.2 Capillary Teleangectasias.- 21.9.3 Cavernous Angioma.- 21.9.4 Arteriovenous Malformation.- 21.9.4.1 Dural Arteriovenous Malformations.- 21.9.5 Venous Malformations.- 22 Phakomatosis and Dysgenetic Syndromes.- 22.1 Tuberous Sclerosis (Bourneville’s Disease).- 22.2 Neurofibromatosis.- 22.2.1 Neurofibromatosis-1 or von Recklinghausen’s Disease.- 22.2.1.1 Clinical Course.- 22.2.2 Neurofibromatosis-2.- 22.2.3 Associated Lesions of a Dysplastic Nature.- 22.3 Von Hippel-Lindau Syndrome.- 22.4 Sturge-Weber Syndrome.- 22.5 Other Dysgenetic Syndromes.- 23 Primary Central Nervous System Lymphomas.- 23.1 Frequency, Age, Site, and Clinical Features.- 23.2 Macroscopic Aspect and Imaging.- 23.3 Microscopic Appearance.- 23.4 Epidural Lymphomas.- 23.5 Lymphomas in AIDS.- 23.6 Prognosis, Treatment.- 24 Metastases.- 24.1 Frequency.- 24.2 Sex.- 24.3 Age.- 24.4 Metastatic Pathways.- 24.5 Macroscopic Appearance and Imaging.- 24.6 Microscopic Appearance.- 24.7 Differential Diagnosis.- 24.8 Prognosis and Therapy.- 24.9 Carcinomatous Meningitis.- 24.10 Spinal Metastases.- 25 Biological Basis of Therapies.- 25.1 Radiotherapy.- 25.1.1 Cellular Response to Ionizing Radiation.- 25.1.2 Therapeutic Studies with Low Linear Energy Transfer Radiation on Experimental Brain Tumors.- 25.1.3 Methods of Improving the Therapeutic Ratio in Radiotherapy of Brain Tumors.- 25.1.3.1 Altered Fractionation.- 25.1.3.2 Brachytherapy.- 25.1.3.3 Association with Chemotherapeutic Agents.- 25.1.3.4 Radiosensitizers.- 25.1.3.5 Hyperthermia.- 25.1.3.6 Photoradiation Therapy.- 25.1.3.7 High Linear Energy Transfer Radiation.- 25.1.3.8 Radioprotectors.- 25.2 Chemotherapy.- 25.2.1 General Concepts.- 25.2.2 Chemosensitivity and Chemoresistance in Brain Tumors.- 25.2.3 Drug Delivery to Brain Tumors.- 25.2.3.1 Intra-Cerebrospinal Fluid and Interstitial Chemotherapy.- 25.2.3.2 Transient and Reversible Blood-Brain Barrier Modification.- 25.2.3.3 Carrier Systems and Liposomes.- 25.3 Immunotherapy.- 25.4 Biologic Therapies.- 26 Effects of Treatment on Brain Tumors and Normal Nervous Tissue.- 26.1 Effects of Radiotherapy and/or Chemotherapy on Human Brain Tumors.- 26.2 Effects of External Radiotherapy on the Human Brain.- 26.3 Effects of Brachytherapy on the Human Brain.- 26.4 Effects of External Radiotherapy on the Human Spinal Cord and/or Nerve Roots.- 26.5 Pathogenesis of Adverse Effects of Radiotherapy on the Normal Nervous Tissue.- 26.6 Effects of Chemotherapy on the Human Brain and Spinal Cord.- 26.7 Effects of Treatment on Normal Nervous Tissue in Acute Lymphocytic Leukemia of Childhood.- 26.8 Second Malignancies.- References.