Brainstem Tumors

Description

Foreword 1 Foreword 2 Preface Acknowledgments Contents Contributors Chapter 1: History of Brainstem Glioma Surgery 1.1 Introduction 1.2 Classical Era 1.3 Fifteenth Through Seventeenth Centuries 1.4 Eighteenth Century 1.5 Nineteenth Century 1.6 Early Twentieth Century 1.7 Mid-to-Late Twentieth Century 1.8 Conclusion References Chapter 2: Anatomy of the Brainstem 2.1 Introduction 2.2 Developmental Anatomy: Phylogeny and Ontogeny 2.3 Anatomical Features of the Brainstem 2.3.1 Mesencephalon (Midbrain) 2.3.2 Pons (Bridge) 2.3.3 Medulla Oblongata 2.4 Vascular Anatomy of the Brainstem 2.4.1 Arterial Supply [7, 8] 2.4.1.1 Midbrain 2.4.1.2 Pons 2.4.1.3 Medulla 2.4.2 Venous Anatomy [9] 2.4.2.1 Midbrain 2.4.2.2 Pons 2.4.2.3 Medulla 2.4.3 Vascular Relationships and the Rule of Three [8] 2.5 Special Microsurgical Regions and Surgical Safe Entry Zones 2.5.1 Interpeduncular Fossa and Cerebral Peduncles [15, 16] 2.5.2 Quadrigeminal and Ambient Cisterns 2.5.3 Floor of Fourth Ventricle [4, 19] 2.5.4 Cerebellopontine Angle and Related Cisterns 2.6 Conclusion References Chapter 3: Imaging of Brainstem Lesions 3.1 Introduction 3.2 Imaging Techniques 3.2.1 Magnetic Resonance Imaging (MRI) 3.2.2 Computed Tomography (CT) 3.2.3 Positron Emission Tomography (PET) 3.2.4 Ultrasound 3.3 Intra-Axial Lesions: Neoplastic 3.3.1 Brainstem Glioma 3.3.1.1 Imaging 3.3.2 Lymphoma 3.3.2.1 Imaging 3.3.3 Metastatic Disease 3.3.3.1 Imaging 3.4 Intra-Axial Lesions: Non-Neoplastic 3.4.1 Cavernous Malformation 3.4.1.1 Imaging 3.4.2 Capillary Telangiectasia 3.4.2.1 Imaging 3.4.3 Hemorrhage/Hematoma 3.4.3.1 Imaging 3.4.4 Infarction 3.4.4.1 Imaging 3.4.5 Hypertrophic Olivary Degeneration 3.4.5.1 Imaging 3.4.6 Demyelination 3.4.6.1 Imaging 3.4.7 Infection 3.4.7.1 Imaging 3.5 Extra-Axial Lesions 3.5.1 Schwannoma 3.5.1.1 Imaging 3.5.2 Meningioma 3.5.2.1 Imaging 3.5.3 Epidermoid Cyst 3.5.3.1 Imaging 3.6 Conclusion References Chapter 4: Clinical Presentation and Assessment for Brainstem Tumors 4.1 Introduction 4.2 Patient History 4.3 Neurological Examination 4.3.1 Cranial Nerve Palsies 4.3.1.1 Trochlear Nerve Palsy 4.3.1.2 Trigeminal Nerve Palsy 4.3.1.3 Abducens Nerve Palsy 4.3.1.4 Facial Nerve Palsy 4.3.1.5 Lower Cranial Nerves Palsy 4.3.2 Pyramidal Tract Signs 4.3.3 Truncal Ataxia 4.3.4 Fundoscopic Changes 4.3.5 Nystagmus 4.4 Brainstem Syndromes Associated with Tumors 4.4.1 Midbrain Syndromes 4.4.2 Pontine Syndromes 4.4.3 Medullary Syndromes 4.5 Conclusion References Chapter 5: Intraoperative Neurophysiological Monitoring During Brainstem Surgery 5.1 Introduction 5.2 Surgery of the Midbrain 5.2.1 Mapping 5.2.1.1 Identification of the Oculomotor Nerve Nuclei at the Level of the Tectal Plate 5.2.1.2 Identification of the Corticospinal Tract at the Level of the Cerebral Peduncle 5.2.2 Monitoring 5.2.2.1 Monitoring of Motor Evoked Potentials 5.2.2.2 Monitoring of Brainstem Auditory Evoked Potentials 5.3 Surgery of the Pons 5.3.1 Mapping 5.3.1.1 Mapping of the Facial Colliculus on the Floor of the Fourth Ventricle 5.3.2 Monitoring 5.3.2.1 Monitoring of the Facial Nerve 5.4 Surgery of the Medulla Oblongata 5.4.1 Mapping 5.4.1.1 Mapping the IX/X, XI and XII Cranial Nerve Nuclei 5.4.2 Monitoring 5.4.2.1 Lower Cranial Nerve Monitoring 5.5 Other Monitoring Techniques in the Brainstem 5.6 Conclusion References Chapter 6: Low-Grade Pediatric Brainstem Gliomas 6.1 Introduction 6.2 Classification 6.3 Symptoms 6.4 Diagnosis and Imaging 6.5 Pathology 6.6 Differential Diagnosis 6.7 Treatment Rationale 6.8 Biopsy Considerations 6.9 Surgical Treatment 6.9.1 Treatment of Hydrocephalus 6.9.2 Tumor Resection 6.9.3 Postoperative Complications 6.10 Adjuvant Oncological Treatments 6.11 Outcome and Prognostic Factors 6.12 Follow-Up Modalities 6.13 Adult Low-Grade Brainstem Tumors 6.14 Conclusion References Chapter 7: High-Grade Tumors of the Brainstem (Except DIPG) 7.1 Adult Malignant Brainstem Tumors 7.1.1 Introduction 7.1.2 Clinical Presentation 7.1.3 Imaging and Histopathological Diagnosis 7.1.3.1 Focal Malignant Brainstem Gliomas 7.1.3.2 Focal Tectal Gliomas 7.1.3.3 Other Brainstem Gliomas 7.1.4 Chemotherapy 7.1.5 Conclusion 7.2 Pediatric Malignant Brainstem Tumors, Excluding Diffuse Intrinsic Pontine Gliomas (DIPG) 7.2.1 Introduction 7.2.2 Clinical Presentation 7.2.3 Imaging and Histopathological Diagnosis 7.2.3.1 High-Grade Gliomas (HGG) 7.2.3.2 Atypical Teratoid Rhabdoid Tumor (ATRT) 7.2.3.3 Embryonal Tumor with Multilayered Rosettes (ETMR) and Historical Central Nervous System Primitive Neuroectodermal Tumor (CNS-PNET) 7.2.4 Treatment and Outcome 7.2.4.1 Atypical Teratoid Rhabdoid Tumor (ATRT) 7.2.4.2 Embryonal Tumor with Multilayered Rosettes (ETMR) and Historical Central Nervous System Primitive Neuroectodermal Tumor (CNS-PNET) 7.2.4.3 High-Grade Gliomas (HGG) 7.2.5 Conclusion References Chapter 8: Diffuse Midline Glioma – Diffuse Intrinsic Pontine Glioma 8.1 Introduction 8.2 Clinical Presentation 8.3 Imaging Characteristics 8.4 Surgical Options 8.4.1 To Biopsy or Not to Biopsy a Potential DIPG? 8.4.2 Tenets for Obtaining a DIPG Biopsy 8.5 Pathology and Molecular Signatures 8.6 Management Strategies 8.6.1 Radiotherapy and Chemotherapy 8.6.2 Targeted Therapy 8.7 Conclusion References Chapter 9: Surgery for Vascular Lesions of the Brainstem 9.1 Introduction 9.2 Natural History 9.3 Presentation and Diagnosis 9.3.1 Clinical Presentation 9.3.2 Imaging 9.4 Regional Management Strategies for Brainstem Vascular Lesions 9.4.1 Midbrain 9.4.1.1 Aneurysms 9.4.1.2 Arteriovenous Malformations (AVMs) 9.4.1.3 Cavernous Malformations (CMs) 9.4.2 Pons 9.4.2.1 Aneurysms 9.4.2.2 Arteriovenous Malformations (AVMs) 9.4.2.3 Cavernous Malformations (CMs) 9.4.3 Medulla 9.4.3.1 Aneurysms 9.4.3.2 Arteriovenous Malformations (AVMs) 9.4.3.3 Cavernous Malformations (CMs) 9.5 Conclusion References Chapter 10: Inflammatory and Infectious Lesions of the Brainstem 10.1 Introduction 10.2 Clinical Presentation 10.3 Diagnostic Approach 10.4 Etiologies 10.4.1 Brainstem Infections 10.4.1.1 Brainstem Abscess 10.4.1.2 Brainstem Encephalitis 10.4.1.3 Postinfectious Brainstem Encephalitis 10.4.1.4 Infectious Brainstem Vasculitis 10.4.2 Brainstem Inflammatory Lesions 10.4.2.1 Idiopathic Inflammatory Demyelinating Diseases of the Brainstem 10.4.2.2 Connective Tissue Diseases (CTDs) and Vasculitis 10.4.2.3 Paraneoplastic Brainstem Encephalitis 10.5 Conclusion References Chapter 11: Surgical Approaches to Mesencephalic (Midbrain) Tumors 11.1 Introduction: Anatomy and Embryology 11.2 Midbrain Lesions 11.2.1 Thalamopeduncular Tumors 11.2.2 Tectal Gliomas 11.2.3 Aqueductal and Periaqueductal Tumors 11.3 Surgical Approaches to Midbrain Tumors 11.3.1 Anterior Midbrain 11.3.1.1 Transchoroidal and Subchoroidal Approaches 11.3.1.2 Peritemporal Approaches 11.3.2 Central Midbrain 11.3.3 Posterior (Dorsal) Midbrain 11.4 Surgical Decision-Making 11.5 Prognosis 11.6 Conclusion References Chapter 12: Surgical Approaches to Pontine Tumors 12.1 Introduction 12.2 Surgical Anatomy of the Pons 12.3 Safe Entry Zones 12.3.1 Peritrigeminal 12.3.2 Supratrigeminal 12.3.3 Lateral Pontine (Middle Cerebellar Peduncle) 12.3.4 Suprafacial Collicular (Suprafacial Triangle) 12.3.5 Infrafacial Collicular (Infrafacial Triangle) 12.3.6 Superior Foveal (Lateral Sulcus Limitans) 12.3.7 Fourth Ventricular Median Sulcal (Interfacial) 12.3.8 Superior Ventral Pontine 12.4 Anterior (Ventral) Pons 12.4.1 Orbito-Fronto-Zygomatic Approach 12.4.1.1 Surgical Setup and Patient Positioning 12.4.1.2 Surgical Corridor(s) and Approach 12.4.2 Endoscopic Endonasal Transclival Approach (Fig. 12.8) 12.4.2.1 Surgical Setup and Patient Positioning 12.4.2.2 Surgical Corridor(s) and Approach 12.5 Anterolateral Pons 12.5.1 Transpetrosal (See Fig. 12.8) 12.5.1.1 Surgical Setup and Patient Positioning 12.5.1.2 Surgical Corridor(s) and Approach 12.5.2 Retrosigmoid 12.5.2.1 Surgical Setup and Patient Positioning 12.5.2.2 Surgical Corridor(s) and Approach 12.6 Posterior (Dorsal) Pons 12.6.1 Median Suboccipital Telovelar/Subvermian; Transvermian 12.6.1.1 Surgical Setup and Patient Positioning 12.6.1.2 Surgical Corridor(s) and Approach 12.7 Conclusion References Chapter 13: Surgical Approaches to Medullary Tumors 13.1 Introduction 13.2 Clinical Presentation 13.3 Neuroradiological Evaluation 13.4 Management Strategies 13.5 Our Patient Series 13.5.1 Patient Selection 13.5.2 Tumor Characteristics 13.5.2.1 Tumor Location and Extent 13.5.2.2 Tumor Entities 13.5.2.3 Molecular Signature 13.5.3 Surgical Strategies 13.5.3.1 Goals of Surgery 13.5.3.2 Timing of Surgery 13.5.3.3 Preoperative Planning and Tumor Exposure 13.5.3.4 Microsurgical Dissection Technique 13.5.3.5 Extent of Tumor Resection 13.5.4 Clinical Outcome 13.5.4.1 Pilocytic Astrocytoma 13.5.4.2 Ganglioglioma 13.5.4.3 Anaplastic Astrocytoma 13.5.4.4 Glioblastoma 13.6 Conclusion References Chapter 14: Radiation and Chemotherapy for Brainstem Tumors 14.1 Introduction 14.2 Treatment of Pediatric Diffuse Intrinsic Pontine Glioma (DIPG) 14.2.1 Radiation Therapy 14.2.1.1 Hyperfractionated Radiotherapy 14.2.1.2 Hypofractionated Radiation Therapy 14.2.1.3 Radiosensitizing Agents 14.2.2 Chemotherapy 14.2.2.1 Empiric Therapies 14.2.2.2 Disease-Specific Targeted Therapy 14.2.2.3 Biologic Agents 14.2.2.4 Different Modes of Delivery 14.3 Treatment of Pediatric Non-DIPG Brainstem Gliomas 14.3.1 Radiation Therapy 14.3.2 Chemotherapy 14.4 Treatment of Adult Brainstem Gliomas 14.4.1 Radiation Therapy 14.4.2 Chemotherapy 14.5 Treatment at Relapse 14.6 Conclusion References Chapter 15: Future Therapies for Malignant Brainstem Tumors 15.1 Introduction 15.2 Obstacles in the Treatment of Malignant Brainstem Tumors 15.2.1 Maximal Safe Cytoreduction Surgery 15.2.2 Blood-Brain Barrier 15.2.3 ATP-Binding Cassette Transporters 15.2.4 Intratumoral Heterogeneity 15.2.5 Immune Privilege and Specialization of the Central Nervous System 15.3 Molecular Characteristics of Malignant Brainstem Tumors 15.4 Drug Delivery 15.4.1 Convection-Enhanced Delivery 15.4.2 Intraarterial Delivery 15.4.3 Manipulating the Blood-Brain Barrier 15.4.3.1 Focused Ultrasound 15.4.3.2 Photodynamic Therapy 15.4.3.3 Photochemical Internalization 15.4.4 Inhibition of ATP-Binding Cassette Transporters 15.4.5 Carriers and Packaging Vehicles 15.4.5.1 Liposomes 15.4.5.2 Nanoparticles 15.5 Signal Transduction Pathway Targeted Therapy 15.6 Modulating Gene Expression Status: Epigenetic Modulators 15.7 Immunotherapy 15.7.1 Therapeutic Antibodies, Radiolabeled Antibodies, and Immunotoxins 15.7.2 Therapeutic Vaccines 15.7.3 Immune Checkpoint Inhibitors 15.7.4 Adoptive Cell Therapies 15.8 Oncolytic Viruses 15.9 Conclusion References Index