Comprehensive management of skull base tumors

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Comprehensive Management of Skull Base Tumors MedOne Access Information Title Page Copyright Dedication Contents Videos Preface Acknowledgments Contributors Section I: General Principles 1 Surgical Anatomy of the Cranial Base: Transcranial and Endonasal Approaches 1.1 Cranial Base Anatomy from the Transcranial Perspective 1.1.1 Anterior Cranial Base 1.1.2 Middle Cranial Base 1.1.3 Posterior Skull Base 1.2 Cranial Base Anatomy from the Endonasal Perspective 1.2.1 The Sphenoid Sinus 1.2.2 The Pituitary Gland and Suprasellar Space 1.2.3 Anterior Cranial Base 1.2.4 The Cavernous Sinus 1.2.5 The Clival and Petroclival Regions 1.2.6 Subdivision of the Ventral Clivus 1.2.7 Petroclival Region and Jugular Foramen 1.3 Conclusion 2 The Pathology of Tumors and Tumorlike Lesions of the Skull Base 2.1 Introduction 2.2 Biopsies and Frozen Sections 2.3 Nonneoplastic and Congenital Lesions 2.3.1 Encephalocele 2.3.2 Nasal Glial Heterotopia 2.3.3 Respiratory Epithelial Adenomatoid Hamartoma 2.3.4 Seromucinous Hamartomas 2.3.5 Ectopic Pituitary Adenoma 2.3.6 Inflammatory Pseudotumor 2.3.7 Sinonasal Polyps 2.3.8 Sinonasal (Schneiderian) Papilloma 2.3.9 Human Papillomavirus 2.3.10 Exophytic Sinonasal (Schneiderian) Papilloma 2.3.11 Cylindrical Cell Papilloma 2.3.12 Inverted Sinonasal (Schneiderian) Papilloma 2.4 Inflammatory and Granulomatous Conditions 2.4.1 Allergic Fungal Sinusitis 2.4.2 Invasive Fungal Sinusitis 2.4.3 Rhinoscleroma 2.4.4 Granulomatosis with Polyangiitis 2.4.5 Histiocytosis-X (Eosinophilic Granuloma) 2.4.6 Myospherulosis 2.5 Malignant Epithelial Neoplasms 2.5.1 Squamous Carcinoma 2.5.2 Sinonasal Undifferentiated Carcinoma 2.5.3 SMARCB1 (INI1)-Deficient Sinonasal Carcinoma 2.5.4 Nasopharyngeal Carcinoma 2.5.5 Nuclear Protein in Testis Carcinoma 2.5.6 Sinonasal Adenocarcinoma 2.5.7 Salivary-Type Adenocarcinomas 2.5.8 Human Papillomavirus–Related Carcinoma with Adenoid Cystic–like Features 2.5.9 Nonsalivary Gland Adenocarcinoma 2.6 Mesenchymal Tumors 2.6.1 Benign Tumors 2.6.2 Lobular Capillary Hemangioma (Pyogenic Granuloma) 2.6.3 Glomangiopericytoma (Sinonasal Hemangiopericytoma) 2.6.4 Nasopharyngeal Angiofibroma 2.6.5 Myxoma 2.6.6 PEComa (Perivascular Epithelioid Cell Tumor) 2.7 Malignant Neoplasms 2.7.1 Rhabdomyosarcoma 2.7.2 Fibrosarcoma 2.7.3 Synovial Sarcoma 2.7.4 Hemangioendothelioma/Epithelioid Hemangioendothelioma 2.7.5 Angiosarcoma 2.7.6 Chordoma 2.7.7 Chondrosarcoma 2.7.8 Osteosarcoma 2.8 Neurogenic Neoplasms 2.8.1 Olfactory Neuroblastoma 2.8.2 Ewing Family Tumors 2.8.3 Adamantinoma-like Ewing Family Tumors 2.9 Neuroendocrine Neoplasms 2.9.1 Neuroendocrine Carcinoma 2.9.2 Carcinoid and Atypical Carcinoid Tumors 2.9.3 Large Cell Neuroendocrine Carcinoma 2.9.4 Small Cell Neuroendocrine Carcinoma 2.9.5 Paraganglioma 2.9.6 Melanoma 2.9.7 Melanotic Neuroectodermal Tumor of Infancy 2.9.8 Biphenotypic Sinonasal Sarcoma 2.9.9 Desmoplastic Small Round Cell Tumor 2.10 Lymphoproliferative Disorders 2.10.1 Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease) 2.10.2 Lymphoma 2.11 Miscellaneous 2.11.1 Teratocarcinosarcoma 2.11.2 Meningioma 2.11.3 Ameloblastoma 2.12 Metastatic Neoplasms 3 Imaging of Skull Base Neoplasms 3.1 Introduction 3.2 Selection of Imaging Modality 3.3 Anterior Cranial Base 3.4 Central Skull Base 3.5 Posterior Cranial Base 3.6 Marrow Space 3.7 Conclusion 4 Head, Neck, and Neuro-otologic Assessment of Patients with Skull Base Tumors 4.1 Introduction 4.2 Physical Examination 4.2.1 Head, Scalp, and Skin Exams 4.2.2 Eye, Orbit, and Eye Movement Exams 4.2.3 Ear Exams 4.2.4 Nose and Nasopharynx Exams 4.2.5 Oral Cavity and Oropharynx Exams 4.2.6 Exams of the Larynx 4.2.7 Neck, Parotid, and Thyroid Exams 4.2.8 Exams of the Cranial Nerves 4.2.9 Gait and Balance Testing 4.3 Audiometric, Vestibular, and Electromyographic Tests 4.3.1 Basic Audiometry 4.3.2 Auditory Brainstem Responses 4.3.3 Otoacoustic Emissions 4.3.4 Electronystagmography 4.3.5 Rotatory Chair Testing 4.3.6 Computerized Dynamic Posturography 4.3.7 Vestibular Evoked Potentials 4.4 Conclusion 5 Anesthesia and Intraoperative Monitoring of Patients with Skull Base Tumors 5.1 Introduction 5.2 Patient Positioning 5.2.1 Supine Position 5.2.2 Prone Position 5.2.3 Park-Bench Position 5.2.4 Lateral Position 5.2.5 Sitting Position 5.3 Complications during Skull Base Procedures 5.3.1 Venous Air Embolism 5.3.2 Pneumocephalus 5.3.3 Macroglossia/Facial Swelling 5.3.4 Cerebrovascular Complications 5.3.5 Arrhythmias 5.3.6 Blood Loss 5.3.7 Peripheral Nerve/Cranial Nerve Injuries 5.4 Monitoring and Anesthesia 5.5 Anesthesia Concerns with Vascular Lesions 5.6 Anesthesia and Neuroprotection 5.7 Anesthesia for Endoscopic Skull Base Surgery 5.8 Postoperative Management 5.9 Conclusion 6 Endoscopic Endonasal Skull Base Surgery 6.1 Principles of Endoscopic Endonasal Surgery 6.2 Indications 6.3 Classification of Endonasal Approaches 6.3.1 Sagittal Plane Modules 6.3.2 Coronal Plane 6.4 Reconstruction 6.5 Limitations 6.5.1 Anatomical 6.5.2 Training 6.6 Complications 6.7 Conclusion 7 Reconstruction of Skull Base Defects 7.1 Introduction 7.2 Goals of Reconstruction 7.3 Types of Defects 7.4 Patient Selection 7.5 Endoscopic Techniques 7.5.1 Acellular and Cellular Grafts 7.5.2 Nasoseptal Flap 7.5.3 Posterior Pedicled Inferior Turbinate Flap 7.5.4 Posterior Pedicled Middle Turbinate Flap 7.6 Endoscopic-Assisted and Open Techniques 7.6.1 Endoscopic-Assisted Pericranial Flap 7.6.2 Temporoparietal Fascial Flap 7.6.3 Palatal Flap 7.6.4 Microvascular Free Flap 7.7 Multilayer Bolster and Closure 7.8 Immediate Postoperative Care 7.9 Lumbar Drains 7.10 Outcomes and Postoperative Complications 7.11 Conclusion 8 Radiotherapy for Skull Base Tumors: Principles and Techniques 8.1 Introduction 8.2 History and Principles of Radiation Oncology 8.2.1 Target Volumes 8.2.2 Alpha/Beta Ratio 8.2.3 Linear Energy Transfer and Relative Biological Effectiveness 8.2.4 Fractionation and Sparing Effect 8.2.5 Altered Fractionation 8.2.6 Biologically Effective Dose 8.2.7 Bragg Peak 8.2.8 Biologically Equivalent Dose 8.2.9 Conformal Radiotherapy 8.2.10 Stereotactic Radiation Therapy 8.2.11 Image-Guided Radiation Therapy 8.3 Principles of Radiation Physics and Radiobiology 8.3.1 Electromagnetic Radiation 8.3.2 Particle Radiation 8.3.3 Interaction with Ionizing Radiation 8.3.4 Radiation Cell Killing 8.3.5 Cell Survival Curve 8.3.6 Linear Quadratic Model 8.3.7 &alpha/&beta Ratio 8.3.8 Fractionation and the Four Rs of Radiobiology 8.4 Radiobiology of Hypofractionation and Skull Base Tumors 8.4.1 Normal Tissue Tolerance of Critical Organs 8.5 Modern Radiation Therapy Techniques for Skull Base Tumors 8.5.1 Advances in Photon Therapy 8.5.2 Advances in Particle Therapy 8.5.3 Advances in Radiotherapy Imaging 8.5.4 Stereotactic Radiation Therapy 8.5.5 Target Delineation 8.5.6 Immobilization, Image Guidance, and Real-Time Motion Tracking 8.5.7 Machine Capability and Stereotactic Radiosurgery Platforms 8.6 Conclusion 9 Clinical Applications of Radiotherapy for Skull Base Tumors 9.1 Introduction 9.2 Normal Tissue Constraints of Critical Organs of the Skull Base 9.3 Pituitary Adenoma 9.3.1 Growth Hormone–Secreting Adenoma 9.3.2 PRL-Secreting Adenoma 9.3.3 Adrenocorticotrophic Hormone–Secreting Adenoma 9.3.4 Stereotactic Radiosurgery for Pituitary Adenoma 9.3.5 Fractionated Stereotactic Radiotherapy 9.4 Acoustic Schwannoma 9.4.1 Modern Stereotactic Radiosurgery Series for Acoustic Schwannoma 9.4.2 Fractionated Radiotherapy and Fractionated Stereotactic Radiotherapy for Acoustic Schwannoma 9.4.3 Long-Term Complications after Acoustic Schwannoma Irradiation 9.5 Meningioma 9.5.1 Conformal Radiotherapy for Skull Base Meningioma 9.5.2 Stereotactic Radiosurgery for Meningioma 9.5.3 Proton Beam Therapy for Skull Base Meningioma 9.6 Craniopharyngioma 9.6.1 Fractionated Radiotherapy for Craniopharyngioma 9.6.2 SRS for Craniopharyngioma 9.7 Paraganglioma 9.7.1 Radiotherapy for Paraganglioma 9.7.2 Fractionated Radiotherapy for Paraganglioma 9.7.3 Stereotactic Radiosurgery and Fractionated Stereotactic Radiotherapy for Paraganglioma 9.8 Chordomas 9.8.1 Radiotherapy for Chordoma 9.8.2 Proton Therapy for Chordoma 9.8.3 Carbon Ion Therapy for Chordoma 9.9 Chondrosarcomas 9.9.1 Radiotherapy for Chondrosarcoma 9.9.2 Particle Therapy for Chondrosarcoma 9.10 Nasopharyngeal Carcinoma 9.10.1 Radiotherapy for Stage T3 to T4 Nasopharyngeal Carcinoma 9.10.2 Proton Therapy for Nasopharyngeal Carcinoma 9.10.3 Proton Therapy for Adenoid Cystic Carcinoma of the Nasopharynx 9.11 Paranasal Sinus Tumor 9.11.1 Squamous Cell Carcinomas 9.11.2 Sinonasal Mucosal Melanoma 9.11.3 Adenoid Cystic Carcinoma 9.11.4 Esthesioneuroblastoma 9.11.5 Sinonasal Undifferentiated Carcinoma 9.12 Skull Base Re-irradiation 9.12.1 Stereotactic Radiotherapy for Skull Base Re-irradiation 9.13 Conclusion 10 Management of Benign Skull Base Tumors in Neuro-oncology: Systemic Cytotoxic and Targeted Therapies 10.1 Introduction 10.2 WHO Grade I Meningioma 10.2.1 Introduction and Treatment 10.2.2 Pathogenesis, Genetics, Molecular, and Cellular Biology 10.2.3 Systemic Therapies 10.3 Chordomas 10.3.1 Introduction to Chordomas and Treatment Overview 10.3.2 Pathogenesis, Genetics, and Molecular and Cellular Pathways 10.3.3 Systemic Therapies 10.4 Craniopharyngioma 10.4.1 Introduction to Craniopharyngiomas and Treatment Overview 10.4.2 Pathogenesis, Genetics, Molecular and Cellular Overview 10.4.3 Systemic Therapies 10.5 Giant Cell Tumor of the Bone 10.5.1 Introduction to Giant Cell Tumor of the Bone and Treatment Overview 10.5.2 Pathogenesis, Genetics, and Molecular and Cellular Pathways 10.5.3 Systemic Therapies 10.6 Conclusion 11 Rehabilitation of Speech and Swallowing of Patients with Tumors of the Skull Base 11.1 Introduction 11.2 Normal Neurophysiology of Swallowing and Communication 11.2.1 Swallowing Neurophysiology 11.2.2 Communication Neurophysiology 11.3 Common Disorders of Swallowing 11.3.1 Anterior Skull Base 11.3.2 Middle Skull Base 11.3.3 Posterior Skull Base 11.4 Common Disorders of Communication 11.4.1 Anterior Skull Base 11.4.2 Middle and Posterior Skull Base 11.5 Assessment and Intervention in Swallowing and Communication Disorders 11.5.1 Clinical Assessment 11.5.2 Instrumental Assessments 11.5.3 Communication Examination 11.5.4 Treatment 11.6 Conclusion 11.7 Case Report 1 11.8 Case Report 2 12 Neuropsychological Assessment of Patients with Skull Base Tumors 12.1 Introduction 12.2 Neurocognitive Impairment Associated with Skull Base Tumors 12.2.1 Tumor-Related Neurocognitive Impairment 12.2.2 Treatment-Related Neurocognitive Impairment 12.3 Affective Distress in Patients Who Have Skull Base Tumors 12.4 Neuropsychological Assessment of Patients Who Have Skull Base Tumors 12.4.1 Clinical Interview 12.4.2 Neurocognitive Test Selection 12.4.3 Mood and Quality of Life Assessment 12.4.4 Care Path 12.5 Management of Neurocognitive and Affective Symptoms in Skull Base Tumors 12.6 Conclusion 13 Cerebrovascular Management in Skull Base Tumors 13.1 Introduction 13.2 Preoperative Imaging 13.3 Preoperative Embolization 13.4 Management of Arterial Encasement 13.4.1 Operative Technique in a Tumor with Subarachnoid Encasement 13.4.2 Decision to Bypass in Cases of Vascular Occlusion or Sacrifice 13.4.3 Choice of Bypass Grafts 13.4.4 Anesthesia, Monitoring, and Preparation 13.4.5 High-Flow (Replacement) Bypass Technique 13.4.6 Low-/Moderate-Flow (Augmentation) Bypass Techniques 13.4.7 Staged Operations 13.4.8 Postoperative Management 13.4.9 Results 13.5 Preservation and Reconstruction of Veins and Sinuses 13.5.1 Venous Reconstruction 13.5.2 Cerebral Venous Sinuses 13.5.3 Intraoperative Sinus Occlusion Test 13.5.4 Reconstruction of Venous Sinuses 13.5.5 Results for Tumors Involving Cerebral Veins and Venous Sinuses 13.5.6 Vasospasm after Cranial Base Tumor Resection 13.6 Case Study 1 13.7 Case Study 2 13.8 Case Study 3 13.9 Case Study 4 13.10 Case Study 5 14 Quality of Life and Measures of Outcome for Patients with Skull Base Tumors 14.1 Introduction 14.2 Challenges in Assessing Quality of Life in Patients Who Have Skull Base Tumors 14.3 Symptom-Specific Quality of Life and Functional Outcomes Following Skull Base Surgery 14.3.1 Nasal Outcomes 14.3.2 Visual Outcomes 14.3.3 Endocrine Outcomes 14.3.4 Neurologic Outcomes 14.4 Disease-Specific Quality of Life Instruments for Skull Base Pathology 14.5 Quality of Life Following Treatment of Skull Base Tumors 14.5.1 Open Surgical Approaches 14.5.2 Endoscopic Endonasal Surgical Approaches 14.5.3 Endoscopic versus Open Surgical Approaches 14.5.4 Radiotherapy 14.6 Conclusion 15 Pediatric Skull Base Surgery 15.1 Pathology 15.1.1 Benign Lesions 15.1.2 Malignant Lesions 15.1.3 Cytogenetic Variations 15.2 Principles of Pediatric Skull Base Surgery 15.2.1 Surgical Techniques 15.2.2 Pediatric Skull Base Reconstruction 15.2.3 Potential Complications 15.3 Conclusion Section II: Site-Specific Considerations 16 Surgical Management of Tumors of the Nasal Cavity, Paranasal Sinuses, Orbit, and Anterior Skull Base 16.1 Introduction 16.2 Surgical Anatomy 16.2.1 Nasal Cavity 16.2.2 Maxillary Sinus 16.2.3 Ethmoid Sinus 16.2.4 Frontal Sinus 16.2.5 Sphenoid Sinus 16.2.6 Infratemporal Fossa 16.2.7 Pterygopalatine Fossa 16.2.8 Anterior Cranial Fossa 16.2.9 Orbit 16.3 Regional Pathology and Differential Diagnosis 16.3.1 Tumors of the Nasal Cavity and Paranasal Sinuses 16.3.2 Tumors of the Orbit 16.4 Clinical Assessment 16.4.1 History and Clinical Examination 16.5 Imaging 16.5.1 Indications 16.5.2 Imaging Modalities 16.6 Biopsy 16.6.1 Nasal Cavity and Paranasal Sinuses 16.6.2 Orbit 16.7 Preoperative Preparation 16.8 Surgery 16.8.1 Indications and Contraindications 16.8.2 Surgical Principles 16.8.3 Surgical Approach 16.8.4 Extent of Resection 16.9 Outcomes and Prognosis 16.10 Conclusion 17 Nasopharyngeal Carcinoma 17.1 Introduction 17.2 Surgical Anatomy 17.3 Regional Pathology and Differential Diagnosis 17.4 Clinical Assessment 17.5 Diagnostic Imaging 17.6 Preoperative Preparation 17.7 Surgical Technique 17.8 Positioning 17.9 Incision 17.10 Soft Tissue Dissection 17.11 Bony Dissection and Osteotomies 17.12 Tumor Resection 17.13 Reconstruction 17.14 Closure 17.15 Postoperative Care 17.16 Complications and Their Avoidance 17.16.1 Complications during the Operation 17.16.2 Postoperative Complications 17.17 Follow-up and Rehabilitation 17.18 Conclusion 18 Clival Tumors 18.1 Introduction 18.2 Surgical Anatomy 18.3 Diagnostic Imaging, Regional Pathology, and Differential Diagnosis 18.4 Clinical Assessment 18.5 Surgical Management 18.6 Transsphenoidal (Microscopic) Approach 18.7 Transsphenoethmoidal Approach 18.8 Transoral–Transpalatal 18.9 Transmaxillary Approaches 18.10 Transmandibular, Circumglossal, Retropharyngeal, Transpalatal Approach to Clivus and Upper Cervical Spine 18.11 Extended Transbasal Approach 18.12 Transpetrous Approaches 18.13 Endonasal Endoscopic Transclival/Transpterygoid Approach 18.13.1 Positioning/Preprocedure Planning 18.13.2 Sinonasal Dissection 18.13.3 Clival Window/Resection 18.13.4 Reconstruction 18.14 Transcondylar Approach 18.15 Anterolateral Transcondylar Approach 18.15.1 Positioning and Incision 18.15.2 Soft Tissue Dissection 18.15.3 Bony Dissection and Osteotomies 18.15.4 Tumor Resection 18.15.5 Reconstruction and Closure 18.16 Posterolateral Transcondylar Approach 18.16.1 Positioning and Incision 18.16.2 Soft Tissue Dissection 18.16.3 Bony Dissection and Osteotomies 18.16.4 Tumor Resection 18.16.5 Reconstruction and Closure 18.16.6 Postoperative Care and Follow-up 19 Tumors of the Infratemporal Fossa 19.1 Introduction 19.2 Surgical Anatomy 19.3 Regional Pathology and Differential Diagnosis 19.4 Clinical Assessment 19.5 Diagnostic Imaging 19.6 Preoperative Preparation 19.7 Surgical Technique 19.7.1 Positioning 19.7.2 Incision 19.7.3 Soft Tissue Dissection 19.7.4 Bony Dissection and Osteotomies 19.7.5 Tumor Resection 19.7.6 Reconstruction 19.7.7 Closure 19.8 Postoperative Care 19.9 Complications and Their Avoidance 19.10 Follow-up and Rehabilitation 19.11 Conclusion 20 Tumors of the Parapharyngeal Space 20.1 Surgical Anatomy 20.2 Regional Pathology and Differential Diagnosis 20.2.1 Salivary Gland Neoplasms 20.2.2 Neurogenic Tumors 20.2.3 Miscellaneous Tumors 20.3 Clinical Assessment 20.4 Diagnostic Imaging 20.5 Preoperative Preparation 20.6 Surgical Technique 20.6.1 Positioning 20.6.2 Incision 20.6.3 Soft Tissue Dissection 20.6.4 Bony Dissection and Osteotomies 20.6.5 Tumor Resection 20.6.6 Closure 20.7 Postoperative Care 20.8 Complications and Avoidance 20.9 Follow-up and Rehabilitation 21 Tumors of the Temporal Bone 21.1 Introduction 21.2 Surgical Anatomy 21.3 Regional Pathology and Differential Diagnosis 21.4 Clinical Assessment 21.5 Diagnostic Imaging 21.6 Preoperative Preparation 21.7 Surgical Technique: Benign Tumors 21.8 Surgical Technique: Malignant Tumors 21.8.1 Operative Preparation and Positioning 21.8.2 Incisions 21.8.3 Bony Dissection and Osteotomies 21.8.4 Soft Tissue Dissection 21.8.5 Reconstruction 21.8.6 Postoperative Care 21.8.7 Complications and Their Avoidance 21.8.8 Follow-up and Rehabilitation 21.9 Conclusion 22 The Evaluation and Management of Sellar Tumors 22.1 Introduction 22.2 Surgical Anatomy 22.2.1 Nasal Cavity 22.2.2 Sphenoid Sinus 22.2.3 Sellar and Parasellar Regions 22.3 Regional Pathology and Differential Diagnosis 22.3.1 Tumors of Pituitary Origin 22.3.2 Tumors of Nonpituitary Origin 22.3.3 Cyst, Hamartomas, and Malformations of the Sellar Region 22.4 Clinical Assessment 22.4.1 Pituitary Hypersecretion 22.4.2 Hypopituitarism 22.4.3 Neurologic Symptoms Secondary to Mass Effect 22.5 Diagnostic Imaging 22.6 Preoperative Preparation 22.7 Surgical Technique 22.7.1 Positioning 22.7.2 Nasal Preparation 22.7.3 Soft Tissue Dissection 22.7.4 Sphenoidotomy and Sellar Entry 22.7.5 Tumor Removal 22.7.6 Reconstruction and Closure 22.8 Postoperative Care 22.9 Complications and Their Avoidance 22.9.1 Sinonasal Complications 22.9.2 Neurologic Complications 22.9.3 Endocrine-Related Complications 22.9.4 Vascular Complications 22.9.5 Cerebrospinal Fluid Leak 22.9.6 Infectious Complications 22.10 Follow-up and Rehabilitation 22.11 Conclusion 23 Tumors of the Cavernous Sinus and Parasellar Space 23.1 History 23.2 Surgical Anatomy 23.3 Regional Pathology and Differential Diagnosis 23.3.1 Meningioma 23.3.2 Trigeminal Schwannoma 23.4 Clinical Assessment 23.5 Diagnostic Imaging 23.6 Preoperative Preparation 23.7 Surgical Technique 23.7.1 Surface Landmarks 23.7.2 Selection of the Approach: Building Block Concept 23.7.3 Positioning 23.7.4 Execution of the Approach 23.8 Tumor Resection 23.9 Closure 23.10 Postoperative Care 23.11 Complications and Their Avoidance 23.12 Follow-up and Rehabilitation 23.13 Conclusion 24 Tumors of the Petrous Apex 24.1 Introduction 24.2 Surgical Anatomy 24.2.1 Regional Pathology and Differential Diagnosis 24.2.2 The Most Frequent Petrous Apex Lesions 24.3 Clinical Assessment 24.3.1 Diagnostic Imaging 24.3.2 Preoperative Preparation 24.3.3 Surgical Approaches 24.4 Translabyrinthine Approach 24.4.1 Indications 24.4.2 Surgical Technique 24.5 Middle Fossa Approach 24.5.1 Indications 24.5.2 Surgical Technique 24.6 Endoscopic Endonasal Approach 24.6.1 Indications 24.6.2 Surgical Technique 24.7 Retrosigmoid Approach 24.7.1 Indications 24.7.2 Surgical Technique 24.8 Presigmoid (Retrolabyrinthine) Approach 24.8.1 Indications 24.8.2 Surgical Technique 24.9 Craniocervical Approach 24.9.1 Indications 24.9.2 Surgical Technique 24.10 Temporal Bone Resection 24.10.1 Indications 24.10.2 Surgical Technique 24.10.3 Postoperative Care 24.10.4 Complications and Avoidance 25 Tumors of the Cerebellopontine Angle 25.1 Introduction 25.2 Imaging 25.2.1 Computed Tomography 25.2.2 Magnetic Resonance Imaging 25.3 Surgical Anatomy 25.4 Treatment Considerations 25.5 Approaches 25.5.1 Retrosigmoid Approach 25.5.2 Translabyrinthine Approach 25.5.3 Middle Fossa Approach 25.6 Complication Avoidance 26 Tumors of the Jugular Foramen 26.1 Anatomical Background 26.2 Regional Pathology and Differential Diagnosis 26.3 Glomus Jugulare Tumors 26.3.1 Pathology 26.3.2 Clinical Presentation 26.3.3 Appearance on Diagnostic Imaging 26.3.4 Preoperative Preparation 26.3.5 Surgical Approach 26.3.6 Postoperative Care 26.3.7 Reconstruction 26.3.8 Results 26.3.9 Other Treatments 26.3.10 Complications 26.3.11 Follow-up and Rehabilitation 26.4 Schwannomas 26.4.1 Pathology 26.4.2 Clinical Presentation 26.4.3 Appearance on Diagnostic Imaging 26.4.4 Preoperative Preparation 26.4.5 Surgical Approach 26.4.6 Postoperative Course 26.4.7 Results 26.4.8 Other Treatments 26.5 Meningiomas 26.5.1 Pathology 26.5.2 Clinical Presentation 26.5.3 Appearance on Diagnostic Imaging 26.5.4 Preoperative Preparation 26.5.5 Surgical Approach 26.5.6 Results 26.5.7 Other Treatments 27 Tumors of the Craniovertebral Junction 27.1 Introduction 27.2 Surgical Anatomy 27.3 Regional Pathology and Differential Diagnosis 27.4 Clinical Assessment 27.5 Diagnostic Imaging 27.6 Preoperative Preparation 27.7 Surgical Technique 27.7.1 Posterior Midline Approach 27.7.2 Far Lateral Approach 27.7.3 Anterolateral Approach 27.7.4 Transoral Approach 27.7.5 Surgical Stabilization 27.8 Postoperative Care 27.9 Complications and Their Avoidance 27.10 Follow-up and Rehabilitation 27.11 Conclusion 28 Tumors of the Orbit 28.1 Introduction 28.2 Osteology 28.3 Regional Pathology and Differential Diagnosis 28.4 Clinical Assessment 28.5 Diagnostic Imaging 28.6 Preoperative Preparation 28.7 Surgical Technique 28.7.1 Extraorbital Transcranial Approaches 28.7.2 Extracranial Transorbital Approaches 28.8 Postoperative Care 28.9 Complications and Their Avoidance 28.10 Conclusion Section III: Tumor-Specific Considerations 29 Squamous Cell Carcinoma of the Nasal Cavity and Paranasal Sinuses 29.1 Epidemiology 29.2 Histopathology 29.3 Disease Spread 29.3.1 Local Spread 29.3.2 Perineural Spread 29.3.3 Regional Metastases 29.3.4 Distant Metastases 29.3.5 Staging 29.4 Treatment 29.4.1 Surgery 29.4.2 Management of the Neck 29.4.3 Radiation Therapy 29.4.4 Chemotherapy 29.4.5 Outcome, Prognosis, and Tumor Biology 30 Nonsquamous Cell Carcinoma of the Nasal Cavity and Paranasal Sinuses 30.1 Introduction 30.2 Adenocarcinoma 30.2.1 Incidence and Epidemiology 30.2.2 Pathology 30.2.3 Clinical Features 30.2.4 Treatment 30.2.5 Outcome and Prognosis 30.3 Salivary Gland Carcinomas 30.3.1 Adenoid Cystic Carcinoma 30.3.2 Non–Adenoid Cystic Carcinoma Salivary Tumors 30.4 Sinonasal Undifferentiated Carcinoma and Neuroendocrine Carcinoma 30.4.1 Incidence and Epidemiology 30.4.2 Pathology 30.4.3 Clinical Features 30.4.4 Treatment 30.4.5 Outcome and Prognosis 30.5 Conclusion 31 Esthesioneuroblastoma 31.1 Incidence and Epidemiology 31.2 Pathology 31.3 Staging 31.4 Treatment 31.4.1 Clinical Features 31.4.2 Imaging 31.4.3 Treatment 31.4.4 Surgery 31.4.5 Radiotherapy 31.4.6 Chemotherapy 31.5 Outcome and Prognosis 31.6 Conclusion 32 Melanoma of the Nasal Cavity and Paranasal Sinuses 32.1 Introduction 32.2 Incidence and Epidemiology 32.3 Pathology 32.4 Clinical Presentation and Findings 32.5 Staging 32.6 Treatment 32.6.1 Surgery 32.6.2 Radiation 32.6.3 Chemotherapy, Immunotherapy, and Checkpoint Inhibitors 32.7 Outcome and Prognosis 32.8 Conclusion 33 Sarcomas of the Skull Base 33.1 Incidence and Epidemiology 33.2 Pathology 33.3 Staging 33.4 Treatment 33.4.1 Surgery 33.4.2 Radiation 33.4.3 Chemotherapy 33.4.4 Doxorubicin 33.4.5 Gemcitabine and Docetaxel 33.4.6 Trabectedin 33.4.7 Eribulin 33.4.8 Pazopanib 33.5 Disease-Specific Considerations 33.5.1 Osteosarcoma 33.5.2 Rhabdomyosarcoma 33.5.3 Solitary Fibrous Tumors 33.6 Conclusion 34 Angiofibromas and Other Vascular Tumors of the Skull Base 34.1 Introduction 34.2 Incidence and Epidemiology 34.3 Pathology 34.3.1 Angiofibroma 34.3.2 Other Vascular Tumors 34.4 Staging 34.5 Treatment 34.5.1 Surgery 34.5.2 Radiation Therapy 34.5.3 Medical Therapy 34.6 Outcome and Prognosis 34.7 Conclusion 35 Chordomas and Chondrosarcomas of the Skull Base 35.1 Introduction 35.2 Epidemiology 35.3 Pathology 35.3.1 Chordomas 35.3.2 Chondrosarcomas 35.4 Staging 35.5 Treatment 35.5.1 Surgery 35.5.2 Radiation 35.5.3 Chemotherapy 35.5.4 Outcome and Prognosis 35.6 Conclusion 36 Meningiomas 36.1 Incidence/Epidemiology 36.2 Etiology 36.2.1 Trauma 36.2.2 Radiation 36.2.3 Infection 36.3 Genetics 36.3.1 Familial Meningioma Syndromes 36.3.2 Chromosomal Abnormalities 36.3.3 Next-Generation Sequencing and NF2 Wild Type Mutations 36.3.4 Gene Sequencing for Prognosis 36.3.5 Genetic Underpinnings of Meningioma Development 36.4 Tumor Biology 36.4.1 Growth Factors 36.4.2 Sex Steroid Receptors 36.4.3 Somatostatin Receptors 36.4.4 Growth Hormone Receptors 36.5 Pathology 36.5.1 WHO Grade I 36.5.2 WHO Grade II 36.5.3 WHO Grade III 36.5.4 Immunohistochemistry 36.6 Treatment 36.6.1 Surgery 36.6.2 Radiation 36.6.3 Chemotherapy 36.6.4 Hormone Therapy 36.6.5 Growth Factor Receptor Inhibitors 36.6.6 Immunotherapy 36.6.7 Targeted Molecular Therapy 36.6.8 Viral Oncolytic Therapy 36.7 Asymptomatic Meningioma 37 Schwannomas of the Skull Base 37.1 Introduction 37.2 Incidence and Epidemiology 37.3 Pathology 37.3.1 Neurofibromatosis Type 2 37.3.2 Mosaicism 37.3.3 Schwannomatosis 37.4 Staging and Presentation 37.4.1 Vestibular Schwannoma 37.4.2 Trigeminal Schwannoma 37.4.3 Facial Schwannoma 37.4.4 Jugular Foramen Schwannoma 37.4.5 Hypoglossal Schwannoma 37.5 Treatment and Outcomes 37.5.1 Vestibular Schwannoma Management 37.5.2 Management of Vestibular Schwannoma in NF2 37.5.3 Management of Trigeminal Schwannoma 37.5.4 Management of Facial Schwannomas 37.5.5 Management of Jugular Foramen Schwanommas 37.5.6 Management of Hypoglossal Schwannomas 38 Paragangliomas of the Head and Neck 38.1 Introduction 38.2 Incidence and Epidemiology 38.2.1 Genetics of Head and Neck Paragangliomas 38.2.2 Malignant Paraganglioma 38.3 Pathology 38.4 Staging 38.5 Treatment 38.5.1 Observation 38.5.2 Surgery 38.5.3 Radiotherapy 38.6 Outcome and Prognosis 38.7 Conclusion 39 Pituitary Adenomas 39.1 Introduction 39.2 Prevalence, Incidence, and Epidemiology 39.3 Pathology 39.3.1 Classification 39.3.2 World Health Organization Grading 39.3.3 Pathogenesis and Biomarkers of Tumor Aggressiveness 39.4 Clinical Presentation, Diagnostic Work-up, and Remission Criteria 39.4.1 Pituitary Incidentaloma and Nonfunctional Pituitary Adenoma 39.4.2 Acromegaly 39.4.3 Cushing's Disease 39.4.4 Prolactinoma 39.4.5 Thyrotropinoma 39.4.6 Pituitary Apoplexy 39.5 Treatment 39.5.1 Conservative Management 39.5.2 Surgery 39.5.3 Medical Therapy 39.5.4 Radiation Therapy 39.5.5 Chemotherapy 39.6 Pituitary Adenoma in Pregnancy 39.7 Outcome and Prognosis 39.7.1 Acromegaly 39.7.2 Cushing's Disease 39.7.3 Prolactinoma 39.7.4 Thyrotropinoma 39.7.5 Pituitary Apoplexy and Nonfunctional Pituitary Adenoma 39.7.6 Follow-up Surveillance Protocol 39.8 Conclusion 40 Craniopharyngiomas 40.1 Introduction 40.2 Incidence and Epidemiology 40.3 Pathology 40.4 Staging 40.5 Preoperative Assessment 40.5.1 Clinical Assessment 40.5.2 Radiographic Assessment 40.6 Postoperative Considerations 40.7 Treatment 40.7.1 Management of Hydrocephalus 40.7.2 Perioperative Endocrine Management 40.7.3 Management of Tumor-Related Cysts 40.7.4 Presence of Calcifications 40.7.5 Relationship to Surrounding Structures 40.7.6 Degree of Lateral Extension 40.8 Surgical Approaches 40.8.1 Transcranial Approaches 40.8.2 Endoscopic Approach 40.9 Adjuvant Therapies 40.9.1 Radiation 40.9.2 Intracavitary Radiation 40.9.3 Chemotherapy 40.9.4 Targeted Therapies 40.10 Outcomes and Prognosis 40.10.1 Comparison of Surgical Approaches 40.10.2 Overall Survival 40.10.3 Morbidities Associated with Surgical Intervention 40.10.4 Overall Risk of Recurrence 40.10.5 Adjuvant Therapy 40.10.6 Management of Recurrence 40.11 Conclusion 41 Epidermoids, Dermoids, and Other Cysts of the Skull Base 41.1 Introduction 41.2 Epidermoids 41.2.1 Incidence and Epidemiology 41.2.2 Embryology 41.2.3 Pathology 41.2.4 Clinical Manifestations 41.2.5 Radiology 41.2.6 Treatment 41.2.7 Outcome and Prognosis 41.3 Dermoids 41.3.1 Incidence and Epidemiology 41.3.2 Embryology 41.3.3 Pathology 41.3.4 Clinical Manifestations 41.3.5 Radiology 41.3.6 Treatment 41.3.7 Outcome and Prognosis 41.4 Cholesterol Granulomas 41.4.1 Incidence and Epidemiology 41.4.2 Pathophysiology 41.4.3 Pathology 41.4.4 Clinical Manifestations 41.4.5 Radiology 41.4.6 Treatment 41.4.7 Outcome and Prognosis 41.5 Arachnoid Cysts 41.5.1 Incidence and Epidemiology 41.5.2 Embryology and Pathophysiology 41.5.3 Pathology 41.5.4 Classification 41.5.5 Clinical Manifestations 41.5.6 Radiology 41.5.7 Treatment 41.5.8 Outcome and Prognosis 42 Fibro-Osseous Lesions of the Skull Base 42.1 Introduction 42.2 Illustrative Case 42.3 Incidence and Epidemiology 42.4 Pathology 42.4.1 Gene Mutation 42.4.2 Histologic Findings 42.4.3 Associated Endocrinopathies 42.4.4 Pain Pathophysiology 42.5 Nonsurgical Management 42.6 Surgical Management 42.6.1 Restoration of Facial Aesthetics 42.6.2 Optic Canal Involvement 42.6.3 Control of Secondary Lesions 42.7 Outcomes and Prognosis 42.8 Secondary Disorders 42.9 Conclusion Index Additional MedOne Access Information