Oncology of CNS Tumors (eBook)

Oncology of CNS Tumors 1
Title Page 2
Copyright Page 3
Preface CNS Textbook,2nd Edition 4
Preface to the 1st Edition 5
Contents 7
Contributors 12
Part I: Cranial Neuro-Oncology 21
Chapter 1 22
Pathology and Classifi cation of Tumors of the Nervous System 22
1.1 Introduction 23
1.1.1 Classification of Tumors of the Nervous System 23
1.1.2 Immunohistochemistry in Brain Tumor Classification 23
1.1.3 Contribution of Molecular Genetics to Brain Tumor Diagnostics 27
1.2 Astrocytic Tumors 28
1.2.1 Diffuse Astrocytoma 29
1.2.2 Anaplastic Astrocytoma 30
1.2.3 Glioblastoma 31
1.2.4 Gliomatosis Cerebri 33
1.2.5 Pilocytic Astrocytoma 34
1.2.6 Pleomorphic Xanthoastrocytoma 35
1.3 Oligodendroglial Tumors and Mixed Gliomas 36
1.3.1 Oligodendroglioma 36
1.3.2 Anaplastic Oligodendroglioma 37
1.3.3 Oligoastrocytoma 38
1.3.4 Anaplastic Oligoastrocytoma 39
1.4 Ependymal Tumors 40
1.4.1 Ependymoma 40
1.4.2 Anaplastic Ependymoma 41
1.4.3 Myxopapillary Ependymoma 41
1.4.4 Subependymoma 42
1.5 Choroid Plexus Tumors 42
1.5.1 Choroid Plexus Papilloma 42
1.5.2 Atypical Choroid Plexus Papilloma 43
1.5.3 Choroid Plexus Carcinoma 43
1.6 Other Neuroepithelial Tumors 43
1.6.1 Astroblastoma 44
1.6.2 Chordoid Glioma of the Third Ventricle 44
1.6.3 Angiocentric Glioma 45
1.7 Neuronal and Mixed Neuronal-Glial Tumors 45
1.7.1 Gangliocytoma and Ganglioglioma 46
1.7.2 Desmoplastic Infantile Astrocytoma/Ganglioglioma 47
1.7.3 Dysembryoplastic Neuroepithelial Tumor 48
1.7.4 Central Neurocytoma and Extraventricular Neurocytoma 48
1.7.5 Cerebellar Liponeurocytoma 49
1.7.6 Papillary Glioneuronal Tumor 49
1.7.7 Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle 50
1.7.8 Paraganglioma 50
1.8 Tumors of the Pineal Region 51
1.8.1 Pineocytoma 51
1.8.2 Pineal Parenchymal Tumor of Intermediate Differentiation 51
1.8.3 Pineoblastoma 52
1.8.4 Papillary Tumor of the Pineal Region 52
1.9 Embryonal Tumors 53
1.9.1 Medulloblastoma 53
1.9.2 Central Nervous System Primitive Neuroectodermal Tumors (CNS-PNET) 55
1.9.2.1 CNS/Supratentorial Primitive Neuroectodermal Tumor 55
1.9.2.2 Medulloepithelioma 56
1.9.2.3 Ependymoblastoma (WHO Grade IV) 56
1.9.3 Atypical Teratoid/Rhabdoid Tumor (WHO grade IV) 57
1.10 Tumors of the Cranial and Paraspinal Nerves 57
1.10.1 Schwannoma 58
1.10.2 Neurofi broma 59
1.10.3 Perineurioma 60
1.10.4 Malignant Peripheral Nerve Sheath Tumor (MPNST) 60
1.11 Meningeal Tumors 61
1.11.1 Meningiomas 61
1.11.2 Mesenchymal, Non-meningothelial Tumors 64
1.11.2.1 Solitary Fibrous Tumor 65
1.11.2.2 Hemangiopericytoma 65
1.11.3 Melanocytic Lesions 66
1.12 Tumors of the Hematopoietic and Lymphoid System 69
1.12.1 Primary Central Nervous System Lymphoma (PCSNL) 69
1.12.2 Histiocytic Lesions Affecting the CNS and Its Coverings 76
1.12.2.1 Langerhans Cell Histiocytosis 77
1.12.2.2 Non-Langerhans Cell Histiocytoses 77
1.13 Germ Cell Tumors of the CNS 78
1.14 Familial Tumor Syndromes 79
1.14.1 Subependymal Giant Cell Astrocytoma 80
1.14.2 Capillary Hemangioblastoma 80
1.14.3 Dysplastic Gangliocytoma of the Cerebellum 82
1.15 Tumors of the Sellar Region 83
1.15.1 Craniopharyngioma (WHO Grade I) 83
1.15.2 Pituitary Adenoma 83
1.15.3 Granular Cell Tumor of the Neurohypophysis 85
1.15.4 Pituicytoma 85
1.15.5 Spindle Cell Oncocytoma of the Adenohypophysis 85
1.16 Metastatic Tumors in the Central Nervous System 86
References 87
Chapter 2 95
Targeted Therapies 95
2.1 Definition 95
2.2 The Basis of Targeting 95
2.3 Targeted Molecules: Growth Factor Systems/Angiogenesis 96
2.4 Targeted Molecules: Signal Transduction 98
2.5 Targeted Molecules: Invasion 98
2.6 Targeted Molecules: The Immune System 99
2.7 Targeted Molecules: Genetic Targeting of Oncolytic Viruses 99
2.8 Targeted Molecules: Radioimmunotherapy with Specific Ligands for “Oncoproteins” 99
2.9 Targeted Delivery: Intraparenchymal Delivery 99
2.10 Targeting Through Motile Delivery Systems 100
2.11 Targeting Other Intracranial Tumors 100
2.12 Targeted Therapies for Meningioma 100
2.13 Targeted Therapies for Ependymoma 100
2.14 Targeted Therapies for Medulloblastoma 100
2.15 Summary 101
References 101
Chapter 3 104
Tumors of the Skull 104
3.1 Epidemiology 104
3.2 Symptoms and Clinical Signs 104
3.3 Diagnostics 105
3.3.1 Synopsis 105
3.4 Staging and Classification 105
3.4.1 Synopsis 105
3.5 Tumors of Bony Origin 105
3.6 Tumors of Cartilaginous Origin 106
3.7 Tumors of Histiocytotic Origin 106
3.8 Fibrous Dysplasia 106
3.9 Miscellaneous 107
3.10 Treatment 108
3.10.1 Synopsis 108
3.10.2 Surgery 108
3.10.3 Radiotherapy 108
3.10.4 Chemotherapy/Medical Therapy 109
3.11 Prognosis/Quality of Life 109
3.12 Follow-Up/Specific Problems and Measures 109
3.13 Future Perspectives 109
References 110
Chapter 4 111
Meningiomas and Meningeal Tumors 111
4.1 Definition 111
4.2 Epidemiology 111
4.3 Molecular Genetics 112
4.4 Etiology and Prevention 112
4.5 Signs and Symptoms 113
4.6 Staging and Classification 114
4.7 Diagnostic Procedures 115
4.8 Therapy 122
4.8.1 Surgery 122
4.9 Prognosis 130
4.10 Follow-Up 130
4.11 Future Perspectives 131
4.12 Other Meningeal Tumors 131
4.12.1 Dural Lymphoma 131
4.12.2 Dural Metastases 131
References 133
Chapter 5 135
Low-Grade Astrocytomas 135
5.1 Epidemiology 135
5.2 Clinical Presentation 136
5.3 Histology and Microscopic Features of Low-Grade Infiltrating Astrocytomas 136
5.3.1 Pathology 136
5.3.2 Microscopic Features 136
5.3.3 Immunohistochemical Features 138
5.3.4 Ultrastructural Features 138
5.4 Conventional Neuroimaging Studies 139
5.5 Emerging Neuroimaging Technologies 139
5.5.1 Magnetic Resonance Imaging 139
5.5.2 Positron Emission Tomography 141
5.5.3 Functional Imaging 141
5.5.4 Magnetoencephalography 141
5.6 Patient Outcome and Survival 141
5.7 Prognostic Factors 142
5.8 Genetic Expression Profile 142
5.9 Treatment Options 143
5.9.1 Observation 143
5.9.2 Surgical Intervention 143
5.9.3 Biopsy 143
5.9.4 Surgical Resection 144
5.9.5 Radiotherapy 146
5.9.6 Chemotherapy 147
5.10 Conclusions 148
References 148
Chapter 6 151
Stereotactic Brachytherapy in Low-Grade Gliomas 151
6.1 Background and Indication 151
6.2 Rationale for Stereotactic Brachytherapy/Radiophysics/Radiobiology 152
6.3 Role of Stereotactic Biopsy 153
6.4 Technique, Implants, and Dosimetry 154
6.5 Follow-Up 155
6.6 Results 155
6.6.1 Adult Glioma Patients 155
6.6.2 Paediatric Glioma Patients 156
6.7 Complications 157
6.7.1 Perioperative Morbidity 157
6.7.2 Risk Estimation of Stereotactic Brachytherapy 158
6.8 Combined Approach (Microsurgery in Combination with Stereotactic Brachytherapy) 158
6.9 Summary 160
References 160
Chapter 7 162
High-Grade Astrocytoma/Glioblastoma 162
7.1 Epidemiology 162
7.2 Symptoms and Clinical Signs 163
7.3 Diagnostics 164
7.3.1 Synopsis 164
7.3.2 Body 165
7.4 Grading and Classification 166
7.4.1 Synopsis 166
7.4.2 Body 167
7.4.3 Treatment 169
7.4.4 Synopsis 169
7.4.5 Body 170
7.4.5.1 Surgery 170
7.4.5.2 Radiotherapy 172
7.4.5.3 Chemotherapy 172
7.4.6 Recurrent Tumors 173
7.5 Prognosis/Quality of Life 173
7.6 Follow-Up/Specific Problems and Measures 174
7.7 Future Perspectives 175
References 175
Chapter 8 177
Oligodendroglioma 177
8.1 Epidemiology 177
8.2 Symptoms and Clinical Signs 177
8.3 Diagnostics 177
8.3.1 Synopsis 177
8.3.2 Body 178
8.4 Staging and Classification 179
8.4.1 Synopsis 179
8.4.2 Body 179
8.5 Treatment 180
8.5.1 Synopsis 180
8.5.2 Body 180
8.5.2.1 Surgery 180
8.5.2.2 Radiotherapy 181
8.5.2.3 Chemotherapy 181
8.5.2.4 Other 181
8.6 Prognosis/Quality of Life 181
8.7 Follow-Up/Specific Problems and Measures 182
8.8 Future Perspectives 182
References 182
Chapter 9 184
Ependymomas and Ventricular Tumors 184
9.1 Defi nition 184
9.2 Epidemiology 184
9.3 Molecular Genetics 185
9.4 Etiology and Prevention 185
9.5 Signs and Symptoms 186
9.6 Staging and Classifi cation 188
9.7 Diagnostic Procedures 188
9.8 Treatment 190
9.8.1 Ependymomas 193
9.9 Follow-Up and Prognosis 195
References 198
Chapter 10 201
Medulloblastoma-PNET, Craniopharyngioma Adult Tumors of Pediatric Origin 201
10.1 Epidemiology 201
10.2 Symptoms and Clinical Signs 202
10.3 Diagnostics 202
10.3.1 Medulloblastoma 202
10.3.2 Craniopharyngioma 202
10.4 Staging and Classification 202
10.4.1 Medulloblastoma 202
10.4.2 Craniopharyngioma 203
10.5 Treatment 203
10.5.1 Medulloblastoma 203
10.5.2 Craniopharyngioma 204
10.6 Prognosis/Quality of Life/Follow-Up/Specific Problems and Measures 205
10.6.1 Medulloblastoma 205
10.6.2 Craniopharyngioma 205
10.7 Future Perspectives 205
References 205
Chapter 11 207
Glioneuronal Tumors 207
11.1 Neuronal and Mixed Neuronal–Glial Tumors 208
11.2 Ganglioglioma and Gangliocytoma 208
11.2.1 Epidemiology 208
11.2.2 Symptoms and Clinical Signs 208
11.2.3 Diagnostics 209
11.2.4 Staging and Classification 210
11.2.5 Treatment 210
11.2.6 Prognosis/Quality of Life 210
11.3 Papillary Glioneuronal Tumor 211
11.3.1 Epidemiology 211
11.3.2 Symptoms and Clinical Signs 211
11.3.3 Diagnostics 211
11.3.4 Staging and Classification 211
11.3.5 Treatment 211
11.3.6 Prognosis/Quality of Life 211
11.4 Desmoplastic Infantile Ganglioglioma/Astrocytoma 211
11.4.1 Epidemiology 211
11.4.2 Symptoms and Clinical Signs 211
11.4.3 Diagnostics 212
11.4.4 Staging and Classification 212
11.4.5 Treatment 212
11.4.6 Prognosis/Quality of Life 212
11.5 Dysplastic Cerebellar Gangliocytoma (Lhermitte–Duclos) 212
11.5.1 Epidemiology 212
11.5.2 Symptoms and Clinical Signs 213
11.5.3 Diagnostics 213
11.5.4 Staging and Classification 213
11.5.5 Treatment 213
11.5.6 Prognosis/Quality of Life 213
11.6 Dysembryoplastic Neuroepithelial Tumor 213
11.6.1 Epidemiology 213
11.6.2 Symptoms and Clinical Signs 214
11.6.3 Diagnostics 214
11.6.4 Staging and Classification 215
11.6.5 Treatment 215
11.6.6 Prognosis/Quality of Life 215
11.7 Central and Extraventricular Neurocytoma 215
11.7.1 Epidemiology 215
11.7.2 Symptoms and Clinical Signs 215
11.7.3 Diagnostics 216
11.7.4 Staging and Classification 216
11.7.5 Treatment 216
11.7.6 Prognosis/Quality of Life 216
11.8 Cerebellar Liponeurocytoma 216
11.8.1 Epidemiology 216
11.8.2 Symptoms and Clinical Signs 216
11.8.3 Diagnostics 217
11.8.4 Staging and Classification 217
11.8.5 Treatment 217
11.8.6 Prognosis/Quality of Life 217
11.9 Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle 217
11.9.1 Epidemiology 217
11.9.2 Symptoms and Clinical Signs 217
11.9.3 Diagnostics 217
11.9.4 Staging and Classification 218
11.9.5 Treatment 218
11.9.6 Prognosis/Quality of Life 218
11.10 Spinal Paraganglioma 218
11.10.1 Epidemiology 218
11.10.2 Symptoms and Clinical Signs 218
11.10.3 Diagnostics 218
11.10.4 Staging and Classification 218
11.10.5 Treatment 219
11.10.6 Prognosis/Quality of Life 219
References 219
Chapter 12 222
Inactive Adenomas 222
12.1 Epidemiology 222
12.2 Symptoms and Clinical Signs 222
12.3 Diagnostics 223
12.4 Classification and Staging 224
12.5 Treatment 225
12.5.1 Surgery 225
12.5.2 Radiotherapy 225
12.5.3 Medical Therapy 226
12.6 Prognosis/Quality of Life 226
12.7 Follow-up/Specific Problems and Measures 227
12.7.1 Incidentalomas 227
12.8 Future Perspectives 227
References 228
Chapter 13 230
Functioning Adenomas 230
13.1 Introduction 230
13.2 Prolactinomas 231
13.2.1 Epidemiology 231
13.2.2 Symptoms and Clinical Signs 231
13.2.3 Diagnostics 231
13.2.3.1 Synopsis 231
13.2.4 Staging and Classification 232
13.2.4.1 Synopsis 232
13.2.5 Treatment 233
13.2.5.1 Synopsis 233
13.2.5.2 Observation 234
13.2.5.3 Medical Treatment 234
13.2.5.4 Surgery 235
13.2.5.5 Radiotherapy 235
13.2.6 Follow-Up/Specific Problems and Measures 235
13.2.6.1 Pregnancy in Women with Prolactinomas 235
13.3 Acromegaly 236
13.3.1 Classification and Epidemiology 236
13.3.2 Symptoms and Clinical Signs 237
13.3.4 Diagnostics 237
13.3.4.1 Synopsis 237
13.3.5 Treatment 238
13.3.5.1 Synopsis 238
13.3.5.2 Surgery 238
13.3.5.3 Medical Treatment 239
13.3.5.4 Radiotherapy 240
13.4 Thyrotropin-Secreting Pituitary Adenomas, Thyrotroph Adenomas 240
13.4.1 Introduction and Epidemiology 240
13.4.2 Symptoms and Clinical Signs 241
13.4.2.1 Endocrinological Evaluation 241
13.4.3 Classification 241
13.4.3.1 Radiological Examination 241
13.4.3.2 Pathological Studies 241
13.4.3.3 Medical Treatment 241
13.4.3.4 Surgery 242
13.4.4 Prognosis/Quality of Life 242
13.4.5 Future Perspectives 242
13.5 Cushing’s Disease 242
13.5.1 Epidemiology 242
13.5.2 Symptoms and Clinical Signs 243
13.5.3 Diagnostics 243
13.5.4 Classification 244
13.5.5 Treatment 244
13.5.5.1 Surgery 245
Transsphenoidal Surgery 245
Transcranial Surgery 245
Bilateral Adrenalectomy 245
13.5.5.2 Radiotherapy 245
Conventional Radiation Therapy 245
Stereotactic Radiosurgery and Fractionated Stereotactic Radiation Therapy 245
Interstitial Brachytherapy 245
13.5.5.3 Pharmacotherapy 246
ACTH-Release Inhibition 246
Inhibition of Steroid Synthesis or Effects at the Receptor 246
13.5.6 Prognosis/Quality of Life 246
13.5.7 Follow-Up/Specific Problems and Measures 246
13.5.8 Future Perspectives 247
References 247
Chapter 14 249
Tumors of the Pineal Region 249
14.1 Epidemiology 249
14.2 Genetics of Pineal Tumors 250
14.3 Symptoms and Clinical Signs 251
14.4 Diagnosis 251
14.5 Staging and Classifi cation 252
14.6 Treatment 253
14.6.1 Surgery 253
14.6.2 Radiotherapy and Chemotherapy 256
14.7 Prognosis/Quality of Life 257
14.8 Follow-Up/Specifi c Problems and Measures 258
14.9 Future Perspectives 258
References 258
Chapter 15 260
Tumors of the Cranial Nerves 260
15.1 Epidemiology 260
15.2 Symptoms and Clinical Signs 261
15.3 Diagnostics 261
15.4 Staging and Classification 262
15.5 Treatment 263
15.5.1 Meningioma Associated with Cranial Nerves 264
15.5.2 Optic Nerve Glioma 264
15.5.3 Schwannomas 265
15.5.3.1 Orbital Cavity Schwannoma 265
15.5.4 Vestibular Schwannoma 265
15.5.4.1 General Aspects 265
Malignant Peripheral Nerve Sheath Tumors 271
15.6 Prognosis/Quality of Life 272
15.7 Follow-Up/Specific Problems and Measures 272
15.8 Future Perspectives 272
References 273
Chapter 16 277
Hemangioblastoma and Von Hippel–Lindau Disease 277
16.1 Hemangioblastoma 277
16.2 Von Hippel–Lindau Disease 277
16.3 VHL Protein 278
16.4 Symptoms and Clinical Signs 278
16.4.1 Hemangioblastoma 278
16.4.2 Hemangioblastoma of the Retina 279
16.4.3 Von Hippel–Lindau Disease 279
16.5 Diagnostics 280
16.5.1 Synopsis 280
16.5.2 Histopathology of Hemangioblastoma 280
16.5.3 Imaging of Hemangioblastoma 281
16.5.4 Diagnosis of Hemangioblastoma of the Retina 281
16.5.5 Diagnosis of Von Hippel–Lindau Disease 281
16.6 Staging and Classifi cation 281
16.7 Treatment 282
16.7.1 Synopsis 282
16.7.2 Microsurgery of Hemangioblastoma 282
16.7.3 Stereotactic Radiotherapy of Hemangioblastoma 282
16.7.4 Treatment of Hemangioblastoma of the Retina 282
16.7.5 Treatment of Hemangioblastomas in VHL 283
16.7.6 Chemotherapy of Hemangioblastoma 283
16.8 Prognosis/Quality of Life 283
16.8.1 Hemangioblastoma 283
16.8.2 Hemangioblastoma of the Retina 283
16.8.3 Von Hippel–Lindau Disease 283
16.9 Follow-Up/Specifi c Problems and Measures 283
16.9.1 Hemangioblastoma 283
16.9.2 Hemangioblastoma of the Retina 284
16.9.3 Von Hippel–Lindau Disease 284
16.10 Future Perspectives 284
16.10.1 Novel Drug Therapies for Hemangioblastoma 284
16.10.2 Novel Drug Therapies of Renal Cell Carcinoma 284
References 284
Chapter 17 286
Tumors of the Skull Base 286
17.1 Introduction 286
17.2 Chordoma and Chondrosarcoma 286
17.2.1 Epidemiology 287
17.2.2 Symptoms and Clinical Signs 287
17.2.3 Diagnostics 288
17.2.4 Staging and Classification 288
17.2.5 Treatment 291
17.2.5.1 Surgery 291
17.2.5.2 Radiotherapy 292
17.2.5.3 Chemotherapy 292
17.2.6 Prognosis/Quality of Life 292
17.2.7 Future Perspectives 293
17.3 Skull-Base Meningiomas 293
17.3.1 Epidemiology 293
17.3.2 Symptoms and Clinical Signs 294
17.3.3 Diagnostics 296
17.3.4 Staging and Classification 298
17.3.5 Treatment 300
17.3.5.1 Meningioma Surgery 300
17.3.5.2 Surgery for Clinoidal and Cavernous Sinus Meningiomas 303
17.3.5.3 Radiotherapy 305
17.3.5.4 Chemotherapy 306
17.4 Paragangliomas 306
17.4.1 Epidemiology 307
17.4.2 Symptoms and Clinical Signs 307
17.4.3 Diagnostics 308
17.4.4 Staging and Classification 309
17.4.5 Treatment 310
17.4.5.1 Surgery 311
17.4.5.2 Radiation 312
17.4.5.3 Chemotherapy 313
17.4.5.4 Embolization 313
17.4.6 Prognosis/Quality of Life 313
References 313
Chapter 18 315
Orbital Tumors 315
18.1 Introduction 315
18.2 Epidemiology 315
18.3 Orbital Anatomy 316
18.3.1 Dimensions 316
18.3.2 Bony Walls 316
18.3.4 Orbital Foramina and Fissures 316
18.3.5 Periorbita and Surgical Spaces 317
18.4 Symptoms and Signs 318
18.5 Clinical History and Orbital Examination 319
18.5.1 History 319
18.5.2 Examination 320
18.5.3 Additional Examinations 321
18.5.4 Imaging 322
18.6 An Approach to Differential Diagnosis 322
18.6.1 Acute Onset 323
18.6.2 Subacute Onset 323
18.6.3 Chronic Onset 323
18.6.4 Acute-on-Chronic Onset 324
18.7 Common Orbital Tumors 325
18.7.1 Benign Orbital Tumors 325
18.7.1.1 Orbital Cystic Tumors 326
18.7.1.2 Orbital Vascular Tumors 327
18.7.1.3 Benign Lacrimal Gland Lesions 327
18.7.1.4 Benign Optic Nerve Tumors 328
18.7.1.5 Benign Tumors of Peripheral Nerve, Bone, or Mesenchyme 328
18.7.2 Malignant Orbital Tumors 330
18.7.2.1 Malignant Orbital Tumors of Childhood 330
18.7.2.2 Orbital Lymphoma in Adults 331
18.7.2.3 Lacrimal Gland Carcinomas 332
18.7.2.4 Secondary Orbital Malignancy from the Eyelids, Paranasal Sinuses, or Globe 333
18.7.2.5 Orbital Metastases in Adults 333
18.7.2.6 Rare Adulthood Malignancies of Mesenchymal or Neural Origin 334
18.8 Principles of Surgical Management 334
18.8.1 Principles of Anterior Orbitotomy 334
18.8.2 Principles of Lateral Orbitotomy 335
18.8.3 Principles of Orbital Exenteration 335
Suggested Reading 336
Chapter 19 337
Primary CNS Lymphoma 337
19.1 Epidemiology 337
19.2 Symptoms and Clinical Signs 338
19.3 Diagnostics 338
19.3.1 Synopsis 338
19.4 Classifi cation and Staging 340
19.4.1 Synopsis 340
19.5 Molecular Pathogenesis 341
19.6 Treatment 341
19.6.1 Synopsis 341
19.6.2 Surgery 341
19.6.3 Radiotherapy 341
19.6.4 Chemotherapy with Radiotherapy 342
19.6.5 Chemotherapy Alone 343
19.6.6 Intrathecal Chemotherapy 343
19.6.7 High-Dose Chemotherapy with Stem-Cell Transplantation 344
19.6.8 Other Therapies 344
19.6.9 Special Problems: The Eye 345
19.7 Treatment of HIV-Positive Patients and Post-Transplant Lymphoproliferation 345
19.8 Prognosis/Quality of Life 345
19.9 Follow-Up/Specifi c Problems and Measures 346
19.10 Therapy of Recurrent Tumor 346
19.11 Future Perspectives 346
References 347
Chapter 20 351
Brain Metastasis 351
20.1 Introduction 351
20.2 Frequency 351
20.3 Incidence 352
20.4 Method of Propagation and Distribution 352
20.5 Clinical Presentation 353
20.6 Radiographic Assessment 353
20.7 Histological Assessment 354
20.8 Prognostic Factors 355
20.9 Specific Treatment Modalities 356
20.9.1 Corticosteroids 356
20.9.2 Radiation Therapy 356
20.9.2.1 Whole-Brain Radiation Therapy 356
20.9.2.2 WBRT with Surgery 356
20.9.2.3 Radiosensitization 357
20.9.2.4 Stereotactic Radiosurgery 357
20.9.2.5 Complications 359
20.9.3 Surgery 360
20.9.3.1 Treatment Goals 360
20.9.3.2 Clinical Decision Making 360
Surgery for Single Brain Metastases 360
Surgery for Multiple Brain Metastases 362
20.9.3.3 Surgical Techniques 363
20.9.3.4 Technological Adjuncts to Surgery 363
20.9.4 Chemotherapy 364
20.10 Conclusion 364
References 365
Part II: Pediatric Neuro-Oncology 368
Chapter 21 369
Neurocutaneous Syndromes 369
21.1 Neurofibromatosis Type I 369
21.1.1 Epidemiology 369
21.1.2 Symptoms and Clinical Signs 369
21.1.3 Diagnosis 371
21.1.4 Treatment 372
21.1.4.1 Surgery 372
21.1.4.2 Radiation Therapy 373
21.1.4.3 Chemotherapy 373
21.1.5 Prognosis and Quality of Life 373
21.1.6 Future Perspectives 373
21.2 Neurofibromatosis Type II 374
21.2.1 Epidemiology 374
21.2.2 Symptoms and Clinical Signs 374
21.2.3 Diagnosis 375
21.2.4 Staging and Classification 375
21.2.5 Treatment 375
21.2.5.1 Surgery 376
21.2.5.2 Radiation Therapy 376
21.2.6 Prognosis and Quality of Life 377
21.3 Tuberous Sclerosis Complex 377
21.3.1 Epidemiology 377
21.3.2 Symptoms and Clinical Signs 377
21.3.3 Diagnosis 379
21.3.4 Treatment 379
21.3.4.1 Surgery 379
21.3.4.2 Other 380
21.3.5 Prognosis and Quality of Life 380
21.3.6 Future Perspectives 380
21.4 Von Hippel–Lindau Disease 380
21.4.1 Epidemiology 380
21.4.2 Symptoms and Clinical Signs 380
21.4.3 Diagnosis 381
21.4.4 Treatment 382
21.4.4.1 Surgery 382
21.4.4.2 Radiation Therapy 382
21.4.5 Prognosis and Quality of Life 383
21.5 Sturge–Weber Syndrome 383
21.5.1 Epidemiology 383
21.5.2 Symptoms and Clinical Signs 383
21.5.3 Diagnosis 384
21.5.4 Treatment 384
21.5.4.1 Surgery 384
21.5.4.2 Other 384
21.5.5 Prognosis and Quality of Life 385
References 385
Chapter 22 389
Supratentorial Hemispheric Low-Grade Gliomas in Children 389
22.1 Epidemiology 389
22.2 Predisposing Conditions 390
22.3 Symptoms and Clinical Signs 390
22.4 Diagnostics 390
22.5 Histopathologic Features 390
22.5.1 Fibrillary Astrocytoma 391
22.5.2 Dysembryoblastic Neuroepithelial Tumors 391
22.5.3 Gangliogliomas 392
22.5.4 Pleomorphic Xanthoastrocytoma 392
22.5.5 Subependymal Giant Cell Astrocytoma 392
22.5.6 Desmoplastic Infantile Gangliogliomas 393
22.6 Imaging Features 393
22.7 Treatment 394
22.8 Natural History 394
22.9 Treatment Strategies 394
22.9.1 Observation 394
22.9.2 Surgery 395
22.9.3 Chemotherapy 395
22.9.4 Radiotherapy 396
22.10 Follow-Up/Specific Problems and Measures 397
References 397
Chapter 23 398
Optic Gliomas 398
23.1 Introduction 398
23.2 Epidemiology 398
23.3 Symptoms and Signs 399
23.4 Diagnostics 399
23.5 Staging and Classification 400
23.6 Treatment 401
23.6.1 Surgery 401
23.6.2 Radiotherapy 402
23.6.3 Chemotherapy 403
23.7 Prognosis/Quality of Life 404
23.8 Follow-Up/Specific Problems and Measures 405
23.9 Future Perspectives 405
References 406
Chapter 24 408
Thalamic Gliomas 408
24.1 Thalamic Tumors in Children 408
24.1.1 Anatomy of the Thalamus 408
24.2 Epidemiology 409
24.3 Symptoms and Clinical Signs 410
24.3.1 Synopsis 410
24.3.2 Body 410
24.4 Diagnosis 410
24.4.1 Synopsis 410
24.4.2 Body 410
24.5 Staging and Classification 412
24.5.1 Synopsis 412
24.5.2 Body 412
24.6 Treatment 414
24.6.1 Synopsis 414
24.6.2 Body 414
24.6.3 Surgery 415
24.6.3.1 Biopsy 415
24.6.3.2 Open Surgery 415
24.6.3.3 Treatment of Hydrocephalus 417
24.6.3.4 Immediate Postoperative Course 417
24.6.4 Radiotherapy 418
24.6.5 Chemotherapy 418
24.7 Prognosis 418
24.8 Follow-Up/Specific Problems and Measures 419
24.9 Future Perspectives 419
References 419
Chapter 25 421
Midbrain Gliomas 421
25.1 Epidemiology 421
25.2 Clinical Symptoms and Signs 421
25.3 Diagnostics 422
25.4 Staging and Classification 423
25.5 Treatment 423
25.5.1 Treatment of Hydrocephalus Caused by Midbrain Tumors 423
25.5.2 Treatment of Midbrain Tumors 425
25.5.3 Radiotherapy 425
25.5.4 Chemotherapy 425
25.6 Prognosis/Quality of Life 425
25.7 Follow-Up/Specific Problems and Measures 427
References 427
Chapter 26 428
Supratentorial High-Grade Gliomas 428
26.1 Epidemiology 428
26.2 Symptoms and Clinical Signs 429
26.3 Diagnostic Studies 429
26.3.1 Computed Tomography 429
26.3.2 Magnetic Resonance Imaging 429
26.3.3 Genetic Features 430
26.4 Staging and Classification 431
26.5 Treatment 432
26.5.1 Surgical 432
26.5.2 Chemotherapy 432
26.5.3 Radiation Therapy 433
26.6 Prognosis and Outcome 433
26.7 New Treatment Strategies 433
References 434
Chapter 27 436
Ganglioglioma 436
27.1 Epidemiology 436
27.2 Genetics 437
27.3 Symptoms and Clinical Signs 437
27.4 Diagnostics 438
27.4.1 Synopsis 438
27.4.2 General Considerations 439
27.4.3 CT 439
27.4.4 MRI 440
27.4.5 Angiography 440
27.4.6 PET 440
27.5 Staging and Classifi cation 441
27.5.1 Synopsis 441
27.5.2 General Considerations 441
27.5.3 Low-Grade Ganglioglioma (WHO Grade I) 441
27.5.4 Anaplastic Ganglioglioma (WHO III) and Ganglioblastoma (WHO IV) 442
27.6 Treatment 442
27.6.1 Synopsis 442
27.6.2 Surgical Treatment 443
27.6.3 Radiotherapy and Chemotherapy 443
27.6.4 Recurrence Rate and Outcome 444
References 444
Chapter 28 446
Cerebellar Astrocytomas 446
28.1 Epidemiology 446
28.2 Symptoms and Clinical Signs 446
28.3 Diagnostics 447
28.3.1 Synopsis 447
28.3.2 Computerized Tomography (CT) 447
28.3.3 Magnetic Resonance Imaging (MRI) 447
28.4 Staging and Classification 448
28.4.1 Synopsis 448
28.4.2 Pathology 448
28.5 Treatment 448
28.5.1 Synopsis 448
28.5.2 Surgery 448
28.5.3 Radiation 450
28.5.4 Chemotherapy 450
28.6 Prognosis/Quality of Life 450
28.7 Follow-Up/Specific Problems and Measures 451
28.8 Future Perspectives 451
References 451
Chapter 29 453
Diffuse Intrinsic Pontine Gliomas 453
29.1 Introduction 453
29.2 Epidemiology 453
29.3 Symptoms and Clinical Signs 454
29.4 Diagnosis 454
29.4.1 Imaging of DIPG 454
29.5 Surgery 455
29.6 Staging/Classification 455
29.7 Treatment 455
29.7.1 Neurosurgery 456
29.7.2 Radiotherapy 456
29.7.3 Chemotherapy 457
29.8 Prognosis 457
29.9 Specific Problems and Measures 457
29.9.1 Steroids 457
29.9.2 Hydrocephalus 458
29.9.3 Bulbar Impairment 458
29.9.4 Analgesia 458
29.9.5 Neurological Disability 458
29.9.6 Family Support 458
29.10 Future Perspectives 458
References 459
Chapter 30 461
Dorsally Exophytic Brain Stem Gliomas 461
30.1 Introduction 461
30.2 Epidemiology 461
30.3 Symptoms and Clinical Signs 461
30.4 Diagnostics 462
30.4.1 CT Scan Findings 462
30.4.2 MR Scan Findings 463
30.5 Staging and Classification 463
30.6 Treatment 464
30.6.1 Surgery 464
30.6.2 Radiotherapy 465
30.6.3 Chemotherapy 465
30.7 Prognosis/Quality of Life 466
30.8 Follow-Up 466
30.9 Future Perspectives 466
References 466
Chapter 31 467
Cervicomedullary Gliomas 467
31.1 Introduction 467
31.2 Symptoms and Signs 468
31.3 Radiologic Diagnosis 468
31.4 Pathology and Growth Patterns 469
31.4.1 Surgical Treatment 470
31.4.2 Radiotherapy 473
31.4.3 Chemotherapy 473
31.5 Outcome/Prognosis/Quality of Life 473
31.6 Follow-Up 474
31.7 Summary 474
References 474
Chapter 32 476
Desmoplastic Infantile Gangliogliomas 476
32.1 Introduction 476
32.2 Epidemiology 476
32.3 Spectrum and Clinical Signs 477
32.4 Diagnostics 477
32.4.1 Imaging Characteristics 477
32.4.1.1 Synopsis 477
32.4.1.2 CT/MRI Characteristics 477
32.5 Staging and Classifi cation 477
32.6 Treatment 478
32.6.1 Synopsis 478
32.6.2 Surgical Treatment 478
32.6.3 Radiotherapy 478
32.6.4 Chemotherapy 478
32.7 Prognosis/Quality of Life 479
32.8 Follow-Up/Specifi c Problems and Measures 479
32.9 Future Perspectives 479
References 479
Chapter 33 481
Pleomorphic Xanthoastrocytoma 481
33.1 Historical Background 481
33.2 Epidemiology 481
33.3 Symptoms and Clinical Signs 482
33.4 Case Presentations 482
33.4.1 Case 1 482
33.4.2 Case 2 483
33.5 Diagnostics 484
33.6 Pathology 484
33.7 Treatment 485
33.8 Prognosis 486
33.9 Future Perspectives 486
References 486
Chapter 34 488
Hypothalamic Hamartoma 488
34.1 Introduction 488
34.2 Pathology 489
34.3 Epidemiology 489
34.4 Clinical Features 490
34.4.1 Epilepsy Characteristics 490
34.4.2 Cognitive Impairment and Behavioral Disturbance 490
34.4.3 Endocrine Disturbance 491
34.4.4 Congenital Malformations Associated with HH 491
34.5 Preoperative Workupand Diagnostics 491
34.5.1 Staging and Classification 491
34.5.2 MRI Features 491
34.6 Treatment 492
34.6.1 Surgical Options for Patients with Intractable Epilepsy 492
34.6.1.1 The Anterior Transcallosal Transseptal Interforniceal Approach 492
34.6.1.2 Pterional or Fronto-Temporal Approach 494
34.6.1.3 Trans-Lamina Terminalis Approach 494
34.6.1.4 Endoscopic Resection or Disconnection 494
34.6.1.5 Stereotactic Radiofrequency Lesioning 494
34.6.2 Radiotherapy 494
34.7 Outcome of Surgery 495
34.7.1 Extent of HH Resection 495
34.7.2 Seizure Outcome 495
34.7.3 Surgical Complications 496
34.7.4 Behavioral and Cognitive Improvements 496
34.7.5 Endocrine Complications 496
34.7.6 Weight and Appetite 496
34.8 Prognosis/Quality of Life 497
34.9 Precocious Puberty 497
34.10 Follow-Up/Specific Problems and Measures 497
34.11 Future Perspectives 497
References 498
Chapter 35 500
Ependymomas 500
35.1 Introduction 500
35.2 Epidemiology 501
35.3 Signs and Symptoms 501
35.4 Diagnostics 502
35.4.1 Magnetic Resonance Imaging 502
35.4.2 Computed Tomography 504
35.5 Pathology 504
35.6 Staging and Classification 505
35.7 Treatment 506
35.8 Surgery 506
35.8.1 Radiation Therapy 507
35.8.2 Chemotherapy 507
35.8.3 Radiosurgery 508
35.9 Prognosis/Quality of Life 508
References 509
Chapter 36 510
Medulloblastoma 510
36.1 Introduction and Epidemiology 510
36.2 Clinical Presentation 510
36.3 Diagnostic Investigations 511
36.4 Surgical and Pathological Classification 513
36.5 Molecular Pathology 515
36.6 Treatment 516
36.6.1 Surgery 516
36.6.2 Radiotherapy 517
36.6.3 Chemotherapy 517
36.7 Prognosis and Quality of Life 518
36.8 Future Perspectives 519
References 519
Chapter 37 521
Supratentorial Primitive Neuroectodermal Tumors 521
37.1 Epidemiology 521
37.2 Symptoms and Clinical Signs 522
37.3 Diagnostics 522
37.3.1 Synopsis 522
37.3.2 Body 522
37.3.2.1 Radiology 522
37.3.2.2 Pathology 524
37.4 Staging and Classification 525
37.4.1 Synopsis 525
37.4.2 Body 525
37.5 Treatment 525
37.5.1 Synopsis 525
37.5.2 Body 525
37.5.2.1 Surgery 525
37.5.2.2 Radiotherapy 526
37.5.2.3 Chemotherapy 526
37.6 Prognosis/Quality of Life 527
37.7 Follow-Up/Specifi c Problems and Measures 527
37.8 Future Perspectives 527
References 528
Chapter 38 529
Dysembryoplastic Neuroectodermal Tumors 529
38.1 Epidemiology 529
38.2 Symptoms and Clinical Signs 530
38.3 Diagnostics 530
38.4 Staging and Classification 530
38.5 Treatment 531
38.5.1 Surgery 531
38.5.2 Radiotherapy 531
38.6 Prognosis/Quality of Life 531
38.7 Follow-Up/Specific Problems and Measures 532
References 532
Chapter 39 534
Meningiomas in Children 534
39.1 Introduction 534
39.2 Epidemiology 534
39.3 Symptoms and Clinical Signs 535
39.4 Diagnostics 535
39.4.1 Synopsis 535
39.5 Staging and Classification 536
39.6 Treatment 536
39.6.1 Synopsis 536
39.6.2 Surgery 536
39.6.3 Radiotherapy 537
39.6.3.1 Conventional Radiotherapy 537
39.6.3.2 Stereotactic Radiosurgery 537
39.7 Prognosis/Quality of Life 537
39.8 Follow-Up 538
39.9 Future Perspectives 538
References 538
Chapter 40 540
Pineal Region Tumors in Children 540
40.1 Epidemiology 540
40.2 Symptoms and Clinical Signs 541
40.3 Diagnostics 541
40.3.1 Synopsis 541
40.3.2 Surgery 541
40.4 Pathology-Based Staging,Classification, Treatment, and Outcome 543
40.4.1 Parenchymal Pineal Tumors 543
40.4.1.1 Pineal Cysts 543
40.4.1.2 Pineocytomas 543
40.4.1.3 Pineal PNETs/Pineoblastomas 543
40.4.1.4 Trilateral Retinoblastomas 543
40.4.2 Glial Pineal Tumors 543
40.4.2.1 Low-Grade Gliomas 543
40.4.2.2 High-Grade Gliomas 544
40.4.2.3 Ependymomas 544
40.4.3 Pineal Germ Cell Tumors 544
40.4.3.1 Germinomas 544
40.4.3.2 Teratomas 544
40.4.3.3 Nongerminomatous Germ Cell Tumors 545
40.4.4 Miscellaneous Pineal Tumors 545
40.4.4.1 Atypical Teratoid/Rhabdoid Tumors (AT/RT) 545
40.4.4.2 Vascular Lesions 545
40.4.4.3 Meningiomas 545
40.5 Follow-Up/Specific Problems and Measures 545
40.6 Future Perspectives 546
References 546
Chapter 41 548
Pituitary Tumors in Children 548
41.1 Epidemiology 548
41.2 Symptoms and Clinical Signs 548
41.3 Diagnostics 549
41.4 Classifi cation 550
41.5 Treatment 550
41.5.1 PRL-Secreting Adenomas 551
41.5.2 Cushing’s Disease 551
41.5.3 GH-Secreting Adenomas 552
41.6 Prognosis 552
41.7 Follow-Up 552
41.8 Future Perspectives 552
References 553
Chapter 42 554
Craniopharyngiomas 554
42.1 Introduction 554
42.2 Epidemiology 554
42.2.1 Prevalence 554
42.2.2 Age 555
42.2.3 Gender 555
42.3 Histogenesis and Pathology 555
42.3.1 Histogenesis 555
42.3.2 Pathology 555
42.4 Symptoms and Signs 555
42.4.1 Ophthalmologic Signs 555
42.4.2 Endocrine Signs 556
42.4.3 Neurological Signs 556
42.5 Diagnostics 557
42.5.1 Plain Skull Radiography 557
42.5.2 CT Scan 557
42.5.3 Magnetic Resonance Imaging (MRI) 557
42.5.4 Angiography 557
42.6 Differential Diagnosis 557
42.7 Staging and Classification 558
42.7.1 Classification in Relation to Sella Turcica and Diaphragma Sellae 558
42.7.2 Classification in Relation to Optic Chiasm 558
42.7.3 Relation to Third Ventricle 559
42.8 Treatment 559
42.8.1 Surgery 559
42.8.2 Radiotherapy 561
42.8.3 Chemotherapy 561
42.9 Prognosis/Quality of Life 562
42.9.1 Extent of Tumor Resection 562
42.9.2 Operative Mortality 562
42.9.3 Tumor Recurrences 563
42.9.4 Functional Outcome 563
42.10 Future Perspectives 564
References 564
Chapter 43 566
Intracranial Germ Cell Tumors 566
43.1 Epidemiology 566
43.2 Symptoms and Clinical Signs 567
43.3 Diagnosis 568
43.3.1 Synopsis 568
43.3.2 Clinical Manifestation 568
43.3.3 Neuro-imaging 568
43.3.4 Tumor Marker 568
43.3.5 Histological Diagnosis 569
43.5.1 Synopsis 575
43.5.2 Germinoma 577
43.5.3 Teratoma 578
43.5.4 Nongerminomatous Germ Cell Tumor 578
43.6 Prognosis/Quality of Life 579
43.7 Follow-Up/Specific Problems and Measures 579
43.8 Future Perspectives 579
References 580
Chapter 44 581
Choroid Plexus Tumors 581
44.1 Epidemiology 581
44.1.1 Symptoms and Clinical Signs 582
44.2 Diagnostic Imaging 582
44.3 Pathological Classification and Staging 584
44.4 Treatment 585
44.4.1 Surgery 585
44.4.2 Radiation Therapy 587
44.4.3 Chemotherapy 588
44.4.4 Angio-Embolization 588
44.5 Prognosis/Quality of Life 588
References 589
Chapter 45 591
Malignant Rhabdoid Tumors of the CNS 591
45.1 Introduction 591
45.2 Epidemiology 591
45.2.1 Incidence 591
45.2.2 Age and Sex Distribution 592
45.2.3 Location 592
45.3 Symptoms and Clinical Signs 592
45.4 Diagnostics 592
45.4.1 Radiological Features 592
45.5 Staging and Classification 593
45.5.1 Molecular Genetics 594
45.6 Treatment 594
45.6 Treatment 594
45.6.1 Surgery 594
45.6.2 Radiotherapy 595
45.6.3 Chemotherapy 595
45.7 Prognosis/Quality of Life 596
45.8 Follow-Up/Specifi c Problems and Measures 596
45.9 Future Perspectives 597
References 598
Chapter 46 600
Langerhans Cell Histiocytosis 600
46.1 Epidemiology 600
46.2 Symptoms and Clinical Signs 601
46.3 Diagnostics 602
46.4 Staging and Classification 604
46.5 Treatment 604
46.5.1 Surgery 605
46.5.2 Radiotherapy 605
46.5.3 Chemotherapy 606
46.6 Prognosis/Quality of Life 606
46.7 Future Perspectives 606
References 607
Chapter 47 608
Tumors of the Skull Base in Children 608
47.1 Introduction 608
47.2 Distinguishing Features of Pediatric Skull Base Tumors 608
47.3 Tumors of the Anterior Cranial Base in Children 609
47.3.1 Fibrous Dysplasia 609
47.3.2 Esthesioneuroblastoma 609
47.4 Neurosurgical Approaches to the Anterior Cranial Base in Children 610
47.5 Tumors of the Middle Cranial Base in Children 612
47.6 Schwannomas 612
47.6.1 Juvenile Nasopharyngeal Angiofibromas 613
47.7 Neurosurgical Approaches to the Middle Cranial Base in Children 613
47.8 Tumors of the Posterior Cranial Base in Children 614
47.8.1 Chordomas and Chondrosarcomas 614
47.8.2 Neurosurgical Approaches to the Posterior Cranial Base in Children 615
47.9 Complications and Outcomes 617
47.10 Conclusions 617
References 618
Chapter 48 622
Tumors of the Cranial Vault in Children 622
48.1 Introduction 622
48.2 Clinical Presentation 622
48.3 Investigation 622
48.4 Specific Lesions 623
48.4.1 Benign Lesions 623
48.4.2 Malignant Lesions 626
48.5 Summary 627
References 628
Chapter 49 629
Epidural Spinal Tumors in Children 629
49.1 Introduction 629
49.2 Epidemiology 629
49.3 Symptoms and Clinical Signs 630
49.4 Diagnostics 631
49.4.1 Synopsis 631
49.5 Staging and Classification 631
49.5.1 Synopsis 631
49.6 Treatment 632
49.6.1 Synopsis 632
49.6.2 Surgery 632
49.6.3 Radiotherapy 633
49.6.4 Chemotherapy 633
49.6.4.1 Neuroblastoma 633
49.6.4.2 Ewing’s Sarcoma 633
49.6.4.3 Rhabdomyosarcoma 633
49.6.4.4 Lymphoma 634
49.7 Prognosis/Quality of Life 634
49.8 Follow-Up 634
49.9 Future Perspectives 634
References 635
Chapter 50 636
Spinal Column Tumors 636
50.1 Introduction 636
50.2 Epidemiology 636
50.3 Symptoms and Clinical Signs 637
50.4 Diagnostics 637
50.5 Staging, Classification, and Terminology 637
50.5.1 Oncologic Staging 637
50.5.2 Benign Tumors 638
50.5.3 Malignant Tumors 638
50.5.4 Surgical Staging 638
50.5.5 Terminology 638
50.6 Benign Tumors 639
50.6.1 Osteoid Osteoma 639
50.6.2 Osteoblastoma 640
50.6.3 Giant Cell Tumors 641
50.6.4 Aneurysmal Bone Cysts 643
50.6.5 Eosinophilic Granuloma 645
50.7 Malignant Tumors 645
50.7.1 Ewing’s Sarcoma 645
50.7.2 Osteogenic Sarcoma 647
50.8 Metastatic Tumors 647
50.9 Spinal Instrumentation 647
50.10 Future Directions 648
References 649
Chapter 51 653
Pediatric Spinal Intradural Extramedullary Tumors 653
51.1 Introduction 653
51.2 Clinical Considerations 654
51.3 Imaging 654
51.4 Surgical Treatment 655
51.5 Prognosis 655
References 656
Chapter 52 657
Intramedullary Spinal Tumors in Children 657
52.1 Epidemiology 657
52.2 Symptoms and Clinical Signs 657
52.3 Diagnostics 658
52.4 Staging and Classifi cation 658
52.5 Treatment 659
52.5.1 Surgery 660
52.5.2 Radiotherapy 662
52.5.3 Chemotherapy 663
52.6 Prognosis/Quality of Life 663
52.7 Follow-Up/Specifi c Problems and Measures 663
52.8 Future Perspectives 663
References 664
Chapter 53 665
Peripheral Nerve Tumors in Children 665
53.1 Epidemiology 665
53.2 Symptoms and Clinical Signs 667
53.3 Diagnostics 669
53.4 Staging, Classifi cation, and Management 669
53.4.1 Neurofi bromas 670
53.4.1.1 Pathology 670
53.4.1.2 Management 671
53.4.2 Neuroblastomas 671
53.4.2.1 Pathology 671
53.4.2.2 Management 671
53.4.3 Rhabdomyosarcoma 672
53.4.3.1 Pathology 672
53.5 Classifi cation, Pathology, and Management of Rare Peripheral Nerve Tumors in Children 673
53.5.1 Schwannomma 673
53.5.2 Perineurioma 673
53.5.3 Malignant Peripheral Nerve Sheath Tumor 674
53.5.3.1 Pathology 674
53.5.3.2 Management 674
53.5.4 Triton Tumors 675
53.6 Future Perspectives 675
References 675
Part III: Spinal Neuro-Oncology 676
Chapter 54 677
Intramedullary Tumors 677
54.1 Defi nition 677
54.2 Epidemiology and Etiology 677
54.3 Histology and Molecular Genetics 678
54.4 Diagnostics 678
54.5 Differential Diagnosis 686
54.5.1 Treatment and Prevention 688
54.6 Prognosis 695
References 695
Chapter 55 697
Intradural Extramedullary Tumors 697
55.1 Epidemiology 697
55.2 Symptoms and Clinical Signs 698
55.3 Diagnostics 698
55.3.1 Synopsis 698
55.3.2 Meningiomas 699
55.3.3 Nerve Sheath Tumors 700
55.4 Staging and Classifi cation 700
55.4.1 Synopsis 700
55.5 Treatment 702
55.5.1 Synopsis 702
55.5.2 Surgery 702
55.5.2.1 Preparation and Positioning 702
55.5.2.2 Monitoring 702
55.5.2.3 Approach 703
55.5.2.4 Ultrasonography 703
55.5.2.5 Tumor Resection 704
55.6 Radiotherapy and Chemotherapy 705
55.7 Prognosis and Functional Outcome 705
55.8 Follow-Up/Specifi c Problems and Measures 705
55.9 Future Perspectives 705
References 706
Chapter 56 707
Epidural Tumors and Metastases 707
56.1 Epidemiology 707
56.2 Symptoms and Clinical Signs 710
56.3 Diagnostics 711
56.3.1 Staging and Classifi cation 713
56.4 Treatment 714
56.4.1 Surgery 714
56.4.2 Radiotherapy 718
56.4.3 Chemotherapy 720
56.4.4 Other Adjuvant Therapies 721
56.5 Prognosis/Quality of Life 722
56.6 Follow-Up/Specifi c Problems and Measures 723
56.7 Future Perspectives 723
References 723
Chapter 57 726
Spinal Robotic Radiosurgery 726
57.1 Introduction 726
57.2 Fiducial-Free Spinal Tracking 727
57.3 Clinical Data 727
57.4 Conclusions 729
References 729
Part IV: Peripheral Nerve Tumors 731
Chapter 58 732
Peripheral Nerve Tumors 732
58.1 Overview and Epidemiology 732
58.2 Symptoms and Clinical Signs 733
58.3 Diagnostics 734
58.4 Staging and Classifi cation 737
58.5 Associated PNT Predisposition 739
58.6 Syndromes 739
58.7 Treatment 740
58.7.1 Overall Comments 740
58.7.2 Specifi c Comments 742
58.7.2.1 Benign Neural PNTs 742
58.7.2.2 Benign Nonneural PNTs 743
58.7.2.3 Malignant Neural PNTs 745
58.7.2.4 Malignant Nonneural PNTs 749
58.8 Conclusions 751
References 751
Part V: Systemic and General Aspects of Neuro-Oncology 753
Chapter 59 754
General Care of Patients with Cancer Involving the Central Nervous System 754
59.1 Introduction 754
59.2 Antineoplastic Therapy for Patients with CNS Malignancies 755
59.2.1 Adjuvant Chemotherapy 755
59.2.2 Chemotherapy for Recurrent Brain Tumors 756
59.2.3 Judging the Effi cacy of Therapy 756
59.2.4 Delivery of Chemotherapy to the Tumor 757
59.2.5 Clinical Trials 758
59.3 Seizures 759
59.4 Brain Edema 760
59.5 Thromboembolic Disease 761
59.6 Psychosocial Issues 761
59.7 Care of the Dying Patient 761
59.8 Conclusions 761
References 762
Chapter 60 765
Palliative Care in Neuro-Oncology 765
60.1 Introduction 765
60.2 Communication 765
60.3 Caring for Patients and Relatives 766
60.4 Organization of Care 766
60.5 Symptom Control 766
60.5.1 Headache 766
60.5.2 Steroids 766
60.5.3 Dysphagia 767
60.5.4 Cognitive and Behavioral Dysfunction 767
60.5.5 Depression 767
60.5.6 Speech and Language Problems 767
60.5.7 Seizures 768
60.5.8 Venous Thromboembolism 769
60.5.9 Mobility Problems 769
60.5.10 Existential Distress 769
60.5.11 Specifi c Issues in the Terminal Phase 769
References 769
Index 771