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Practical Surgical Neuropathology: A Diagnostic Approach E-Book

Practical Surgical Neuropathology: A Diagnostic Approach E-Book

Arie Perry | Daniel J. Brat

(2017)

Additional Information

Book Details

Abstract

Part of the in-depth and practical Pattern Recognition series, Practical Surgical Neuropathology, 2nd Edition, by Drs. Arie Perry and Daniel J. Brat, helps you arrive at an accurate CNS diagnosis by using a pattern-based approach. Leading diagnosticians in neuropathology guide you from a histological (and/or clinical, radiologic, and molecular) pattern, through the appropriate work-up, around the pitfalls, and to the best diagnosis. Almost 2,000 high-quality illustrations capture key neuropathological patterns for a full range of common and rare conditions, and a "visual index" at the beginning of the book directs you to the exact location of in-depth diagnostic guidance.

  • Instructive algorithms provide detailed guidance based on 8 major (scanning magnification) patterns and 20 minor (high magnification) patterns – helping you narrow the range of diagnostic possibilities.
  • A user-friendly design color-codes patterns to specific entities, and key points are summarized in tables, charts, and graphs so you can quickly and easily find what you are looking for.
  • Sweeping content updates keep you at the forefront of recent findings regarding gliomas, embryonal neoplasms, meningiomas, pituitary region and pineal tumors, epilepsy pathology, peripheral nerve sheath tumors, neurodegenerative disorders, tumor predisposition syndromes, and much more.

Improved pattern call-outs are now linked directly within the chapter, reinforcing the patterns for more efficient and complete understanding.


Table of Contents

Section Title Page Action Price
Front Cover cover
IFC_Expert Consult ad with PIN code IFC1
Half title page i
Pattern Recognition Series ii
Practical Surgical Neuropathology: A Diagnostic Approach iii
Copyright Page iv
Contributors v
Series Preface ix
Preface xi
Acknowledgments xiii
Table Of Contents xv
Practical Surgical Neuropathology Major Patterns xvii
1 Neuropathology Patterns and Introduction 1
Abstract 1.e1
Keywords 1.e1
CNS Tumor Classification Schemes and Additional “Neuropathology Patterns” 1
Electron Microscopy 3
Immunohistochemistry 3
Glial Markers 3
Neuronal Markers 3
Epithelial and Meningothelial Markers 15
Proliferation Markers 15
Molecular Diagnostics and Surrogate Molecular Immunostains 15
References 16
2 Normal Brain Histopathology 19
Abstract 19.e1
Keywords 19.e1
Introduction 19
Cell Types 19
Neurons 19
Glia 21
Astrocytes 21
Oligodendrocytes 24
Ependyma 24
Choroid Plexus 25
Microglia 25
Blood Vessels 26
Meningothelial Cells 26
Melanocytes 26
Tissue Organization 26
Cerebral Cortex 26
White Matter 27
Basal Ganglia 27
Thalamus 28
Hippocampus 28
Pineal Gland 29
Pituitary Gland 30
Cerebellum 30
Brainstem 32
Midbrain 32
Pons 33
Medulla 33
Spinal Cord 33
Meninges 33
Peripheral Nerve, Schwann Cells, and Dorsal Root Ganglia 34
Features of Infancy and Childhood 34
Features of the Aging Nervous System 36
Suggested Readings 37
3 Intraoperative Consultation and Optimal Processing 39
Abstract 39.e1
Keywords 39.e1
Types of Neurosurgical Specimens 39
Intraoperative Cytologic Preparations as a Complement to Frozen Tissue Sections 40
Fixation and Staining Options for Intraoperative Cytologic Preparations 40
Frozen Sectioning of Central Nervous System Tissue 41
Artifacts 41
Iatrogenically Introduced Hemostatic and Embolic Agents 44
Intraoperative Evaluation of Margins for Brain Neoplasms 45
Intraoperative Evaluation of Transmissible Spongiform Encephalopathies (Prion Disease) 49
The Bottom Line: What Does the Surgeon Need to Know? 50
Suggested Readings 50
References 50
4 Neuroradiology 53
Abstract 53.e1
Keywords 53.e1
Introduction 53
Basic Noninvasive Diagnostic Imaging Techniques 53
Advanced Noninvasive Diagnostic Imaging Techniques 57
Basic Invasive Diagnostic Imaging Techniques 60
Advanced Invasive Therapeutic Techniques 62
Imaging Patterns in Neuroradiology 62
Pattern 1—The Intracranial Mass 64
Pattern 2—The Calcified Mass 64
Pattern 3—The Cystic Mass 65
Pattern 4—The Hemorrhagic Mass 66
Pattern 5—The Rim-Enhancing Mass 67
Advanced Strategies of Lesion Analysis 67
Conclusion 67
References 68
5 Integrating Molecular Diagnostics With Surgical Neuropathology 71
Abstract 71.e1
Keywords 71.e1
Immunohistochemical Assessment of Genetic Alterations 71
Mutant-Specific Antibodies That Recognize Hotspot Mutations in Oncoproteins 71
Antibodies Against Tumor Suppressor Proteins Commonly Lost Due to Deletion or Truncating Mutations 75
Antibodies Against Oncoproteins That Aberrantly Localize to the Nucleus Based on Genetic Alterations 75
Antibodies Against Proteins That Serve as Surrogate Markers of Defining Genetic Alterations in Tumors 77
Sanger Sequencing for Detection of Single Nucleotide Mutations 77
FISH for Detection of Copy Number Alterations 79
FISH and Reverse Transcription PCR for Detection of Gene Fusions and Other Structural Variants 80
Detection of Recurrent Gene Fusions by FISH 81
Detection of Recurrent Gene Fusions and Other Rearrangements by Reverse Transcription PCR 82
Genome-Wide Copy Number Profiling 82
Next-Generation DNA Sequencing 84
DNA Methylation Profiling 86
MGMT Promoter Methylation Testing in Malignant Gliomas 86
Genome-Wide Methylation Profiling for Molecular Subtyping of CNS Tumors 87
Conclusion 87
Suggested Readings 88
References 88
6 Astrocytic and Oligodendroglial Tumors 91
Abstract 91.e1
Keywords 91.e1
Introduction and Brief Historical Overview 91
Diffuse Astrocytomas 91
Definitions and Synonyms 91
Incidence and Demographics 92
IDH-Mutant Astrocytomas 92
IDH-Wildtype Astrocytomas 92
Pediatric (and Young Adult) Diffuse Astrocytomas 92
Clinical Manifestations and Localization 92
Radiologic Features and Gross Pathology 92
Histopathology 97
Frozen Section Diagnosis of Diffuse Gliomas 99
Histologic Variants and Grading 99
Grading 110
Differential Diagnosis 110
Ancillary Diagnostic Studies 111
Genetics 112
Diffuse Astrocytomas, IDH-Mutant 112
Diffuse Astrocytomas, IDH-Wildtype 112
Pediatric Diffuse Gliomas 113
MGMT Testing 113
Treatment and Prognosis 113
Oligodendroglioma 113
Brief Historical Overview 113
Definitions and Synonyms 114
Incidence and Demographics 114
Clinical Manifestations and Localization 114
Radiologic Features and Gross Pathology 114
Histopathology 114
Histologic Variants and Grading 116
Differential Diagnosis 118
Ancillary Diagnostic Studies 118
Genetics 119
Pediatric Oligodendrogliomas 120
Treatment and Prognosis 120
Oligoastrocytoma, NOS 120
Definitions and Synonyms 120
Histopathology 121
Histologic Variants and Grading 121
Suggested Readings 121
References 121
7 Nondiffuse Astrocytoma Variants 125
Abstract 125.e1
Keywords 125.e1
Pilocytic Astrocytoma 125
Definitions and Synonyms 125
Incidence and Demographics 125
Clinical Manifestations and Localization 125
Radiologic Features and Gross Pathology 125
Histopathology 126
Histologic Variants and Grading 126
Differential Diagnosis 132
Ancillary Diagnostic Studies 133
Genetics 133
Treatment and Prognosis 133
Subependymal Giant Cell Astrocytoma 134
Definitions and Synonyms 134
Incidence and Demographics 134
Clinical Manifestations and Localization 134
Radiologic Features and Gross Pathology 134
Histopathology 135
Differential Diagnosis 135
Ancillary Diagnostic Studies 136
Genetics 137
Treatment and Prognosis 137
Pleomorphic Xanthoastrocytoma 137
Definitions and Synonyms 137
Incidence and Demographics 137
Clinical Manifestations and Localization 137
Radiologic Features and Gross Pathology 137
Histopathology 137
Histologic Variants and Grading 140
Differential Diagnosis 140
Ancillary Diagnostic Studies 140
Genetics 140
Treatment and Prognosis 141
Suggested Readings 142
References 142
8 Ependymomas and Choroid Plexus Tumors 145
Abstract 145.e1
Background 145.e1
Design 145.e1
Outcome 145.e1
Conclusion 145.e1
Keywords 145.e1
Definitions and Synonyms 145
Brief Historical Overview 145
Ependymal Tumors 145
Incidence and Demographics 145
Localization and Clinical Manifestations 146
Radiologic Features and Gross Pathology 146
Histopathology 146
Histologic Variants and Grading 146
Subependymoma (WHO Grade I) 146
Myxopapillary Ependymoma (WHO Grade I) 147
Ependymoma (WHO Grade II) 148
Anaplastic Ependymoma (WHO Grade III) 151
Other Patterns 153
Differential Diagnosis 153
Ancillary Diagnostic Studies 154
Immunohistochemistry 154
Electron Microscopy 155
Genetics 155
Treatment and Prognosis 157
Choroid Plexus Tumors 157
Incidence and Demographics 157
Localization and Clinical Manifestations 158
Radiologic Features and Gross Pathology 158
Histopathology 158
Histologic Variants and Grading 158
Choroid Plexus Papilloma (WHO Grade I) 158
Atypical Choroid Plexus Papilloma (WHO Grade II) 159
Choroid Plexus Carcinoma (WHO Grade III) 159
Differential Diagnosis 159
Ancillary Diagnostic Studies 162
Immunohistochemistry 162
Electron Microscopy 164
Genetics 164
Treatment and Prognosis 164
Other Choroid Plexus Tumors 164
Suggested Readings 165
References 165
9 Other Glial Neoplasms 171
Abstract 171.e1
Keywords 171.e1
Introduction 171
Angiocentric Glioma 171
Brief Historical Overview 171
Definitions and Synonyms 171
Incidence and Demographics 171
Clinical Manifestations and Localization 171
Radiologic Features and Gross Pathology 171
Histopathology 171
Differential Diagnosis 173
Ancillary Diagnostic Studies 173
Genetics 175
Treatment and Prognosis 175
Astroblastoma 175
Brief Historical Overview 175
Definitions and Synonyms 175
Incidence and Demographics 175
Clinical Manifestations and Localization 175
Radiologic Features and Gross Pathology 175
Histopathology 175
Histologic Variants and Grading 176
Differential Diagnosis 178
Ancillary Diagnostic Studies 178
Genetics 178
Treatment and Prognosis 178
Chordoid Glioma 178
Brief Historical Overview 178
Definitions and Synonyms 178
Incidence and Demographics 178
Clinical Manifestations and Localization 179
Radiologic Features and Gross Pathology 179
Histopathology 179
Differential Diagnosis 181
Ancillary Diagnostic Studies and a Histogenetic Hypothesis 181
Genetics 181
Treatment and Prognosis 181
Suggested Readings 181
References 181
10 Neuronal and Glioneuronal Neoplasms 183
Abstract 183.e1
Keywords 183.e1
Brief Historical Overview 183
Gangliogliomas and Gangliocytomas (“Ganglion Cell Tumors”) 183
Definitions and Synonyms 183
Incidence and Demographics 183
Clinical Manifestations and Localization 183
Radiologic Features and Gross Pathology 184
Histopathology 185
Histologic Variants and Grading 187
Differential Diagnosis 188
Ancillary Diagnostic Studies 190
Genetics 190
Treatment and Prognosis 191
Desmoplastic Infantile Astrocytoma and Ganglioglioma 191
Definitions and Synonyms 191
Incidence and Demographics 191
Localization and Clinical Manifestations 191
Radiologic Features and Gross Pathology 191
Histopathology 191
Differential Diagnosis 191
Ancillary Diagnostic Studies 193
Genetics 193
Treatment and Prognosis 193
Dysplastic Gangliocytoma of the Cerebellum (Lhermitte-Duclos Disease) 193
Definitions and Synonyms 193
Incidence and Demographics 193
Clinical Manifestations and Localization 193
Radiologic Features and Gross Pathology 193
Histopathology 195
Differential Diagnosis 195
Ancillary Diagnostic Studies 195
Genetics 195
Treatment and Prognosis 196
Central Neurocytoma and Other Neurocytic Neoplasms 196
Definitions and Synonyms 196
Incidence and Demographics 196
Localization and Clinical Manifestations 196
Radiologic Features and Gross Pathology 196
Histopathology 196
Histologic Variants and Grading 199
Differential Diagnosis 199
Ancillary Diagnostic Studies 200
Immunohistochemistry 200
Proliferative Activity 200
Genetics 200
Treatment and Prognosis 200
Cerebellar Liponeurocytoma 200
Definitions and Synonyms 200
Incidence and Demographics 201
Localization and Clinical Manifestations 201
Radiologic Features and Gross Pathology 201
Histopathology 201
Differential Diagnosis 201
Ancillary Diagnostic Studies 202
Genetics 202
Treatment and Prognosis 202
Dysembryoplastic Neuroepithelial Tumor 202
Definitions and Synonyms 202
Incidence and Demographics 202
Localization and Clinical Manifestations 202
Radiologic Features and Gross Pathology 202
Histopathology 202
Histologic Variants and Grading 205
Differential Diagnosis 205
Ancillary Diagnostic Studies 205
Genetics 205
Treatment and Prognosis 205
Papillary Glioneuronal Tumor 206
Definitions and Synonyms 206
Brief Historical Overview 206
Incidence and Demographics 206
Localization and Clinical Manifestations 206
Radiologic Features and Gross Pathology 206
Histopathology 206
Histologic Variants and Grading 206
Differential Diagnosis 206
Ancillary Diagnostic Studies 206
Genetics 206
11 Pineal Parenchymal Tumors 219
Abstract 219.e1
Keywords 219.e1
Introduction, Definitions, and Synonyms 219
Brief Historical Overview 219
Incidence and Demographics 220
Localization and Clinical Manifestations 220
Radiologic Features and Gross Pathology 220
Histopathology 220
Histologic Variants and Grading 223
Principles of Diagnosis and Grading on Small or Artifact-Ridden Samples 226
Differential Diagnosis 226
Pineal Cyst/Normal Pineal Gland 226
Ependymoma 227
Oligodendroglioma and Neurocytoma 227
Papillary Tumor of the Pineal Region (PTPR) 227
Embryonal Tumors: Atypical Teratoid/Rhabdoid and Embryonal Tumor With Multilayered Rosettes 227
High-Grade Gliomas 227
Ancillary Diagnostic Studies 229
Immunohistochemical Studies 229
Ultrastructural Studies 229
Genetics 229
Cytogenetic Considerations 229
Pineocytomas. 229
Pineoblastomas. 230
Genetic Alterations 230
Genetic Susceptibility 230
Gene Expression 230
Treatment and Prognosis 230
Treatment of Pineocytomas (WHO Grade I) 230
Treatment of Pineoblastomas (WHO Grade IV) 230
Treatment of Pineal Parenchymal Tumors of Intermediate Differentiation (WHO Grades II or III) 230
Prognosis 231
Suggested Readings 231
References 231
12 Embryonal Neoplasms of the Central Nervous System 233
Abstract 233.e1
Keywords 233.e1
Definition and Synonyms 233
Brief Historical Overview 233
Medulloblastoma 234
Definition and Synonyms 234
Incidence and Demographics 235
Localization and Clinical Manifestations 235
Radiologic Features and Gross Pathology 236
Histopathology 236
Histologic Variants and Grading 238
Desmoplastic/Nodular Medulloblastoma 238
Medulloblastoma With Extensive Nodularity 240
Large Cell/Anaplastic Medulloblastoma 240
Medulloblastomas With Myogenic or Melanotic Differentiation 243
Genetically Defined Medulloblastoma Groups 243
Medulloblastoma, WNT Activated 244
Medulloblastoma, SHH Activated 244
Medulloblastoma, Non-WNT/Non-SHH 245
Differential Diagnosis 245
Ancillary Diagnostic Studies 245
Treatment and Prognosis 246
Embryonal Tumor With Multilayered Rosettes (ETMR), C19MC Altered 246
Definition and Synonyms 246
Incidence and Demographics 246
Localization and Clinical Manifestations 246
Radiologic Features and Gross Pathology 246
Histopathology 246
Differential Diagnosis 248
Ancillary Diagnostic Studies 249
Genetics 249
Treatment and Prognosis 249
Atypical Teratoid/Rhabdoid Tumor 249
Definition and Synonyms 249
Brief Historical Overview 250
Incidence and Demographics 250
Localization and Clinical Manifestations 250
Radiologic Features and Gross Pathology 251
Histopathology 251
Differential Diagnosis 252
Ancillary Diagnostic Studies 252
Genetics 252
Treatment and Prognosis 254
Other CNS Embryonal Tumors 254
Definition and Synonyms 254
Incidence and Demographics 254
Localization and Clinical Manifestations 254
Radiologic Features and Gross Pathology 254
Histopathology 254
Histologic Variants and Grading 255
Differential Diagnosis 256
Ancillary Diagnostic Studies 256
Genetics 256
Treatment and Prognosis 256
Suggested Readings 256
References 256
13 Meningiomas 259
Abstract 259.e1
Keywords 259.e1
Introduction and Proposed Etiologies 259
Definition and Analogies With Meningothelial Cells 259
Brief Historical Overview of Nomenclature and Histogenetic Notions 260
Incidence and Demographics 260
Localization and Clinical Manifestations 260
Localization 260
Clinical Manifestations 262
Multiple Meningiomas 263
Radiologic Features and Gross Pathology 263
Radiology and Cerebral Edema 263
Gross Pathology 264
Histopathology 266
Histologic Variants and Grading 267
Meningiomas That Are Mostly Benign (WHO Grade I) 268
Meningothelial Meningioma 268
Fibrous (Fibroblastic) Meningioma 268
Transitional Meningioma 268
Psammomatous Meningioma 268
Angiomatous (Vascular) Meningioma 268
Microcystic Meningioma 269
Secretory Meningioma 269
Lymphoplasmacyte-Rich Meningioma 270
Metaplastic Meningiomas 273
WHO Grade II Meningiomas 273
Atypical Meningioma 273
Clear Cell Meningioma 275
Chordoid Meningioma 277
Brain Invasive Meningioma 278
WHO Grade III (Malignant) Meningiomas 280
Anaplastic (Malignant) Meningioma 280
Papillary Meningioma 280
Rhabdoid Meningioma 284
Other Meningioma Patterns Not Included in the WHO 284
Metastasis of and to Meningiomas 287
Differential Diagnosis 289
Ancillary Diagnostic Studies 292
Immunohistochemistry 292
Hormone Studies 293
Electron Microscopy 294
Genetics 294
Cytogenetics 294
Molecular Genetics 294
Treatment and Prognosis 296
Suggested Readings 296
References 296
14 Mesenchymal Tumors of the Central Nervous System 299
Abstract 299.e1
Keywords 299.e1
Introduction 299
Brief Historical Overview 299
Incidence and Demographics 300
Adipocytic Tumors, Including Lipoma, Angiolipoma, and Liposarcoma 300
Definition and Synonyms 300
Localization and Clinical Manifestations 300
Radiologic Features and Gross Pathology 300
Histopathology 300
Histologic Variants and Grading 301
Differential Diagnosis 301
Ancillary Diagnostic Studies 301
Genetics 301
Treatment and Prognosis 301
Bone Tumors, Including Osteoma, Osteoid Osteoma/Osteoblastoma, and Osteosarcoma 301
Definition and Synonyms 301
Localization and Clinical Manifestations 301
Radiologic Features and Gross Pathology 302
Histopathology 302
Histologic Variants and Grading 302
Differential Diagnosis 302
Ancillary Diagnostic Studies 302
Genetics 302
Treatment and Prognosis 303
Cartilaginous Tumors, Including Chondroma and Chondrosarcoma 303
Definition and Synonyms 303
Localization and Clinical Manifestations 303
Radiologic Features and Gross Pathology 303
Histopathology 303
Histologic Variants and Grading 303
Differential Diagnosis 303
Ancillary Diagnostic Studies 305
Genetics 305
Treatment and Prognosis 305
Notochordal Tumors, Including Benign Notochordal Cell Tumor and Chordoma 305
Definition and Synonyms 305
Localization and Clinical Manifestations 305
Radiologic Features and Gross Pathology 305
Histopathology 305
Histologic Variants and Grading 305
Differential Diagnosis 307
Ancillary Diagnostic Studies 307
Genetics 307
Treatment and Prognosis 307
Fibroblastic/Myofibroblastic Tumors, Including Solitary Fibrous Tumor and Inflammatory Myofibroblastic Tumor 307
Definition and Synonyms 307
Localization and Clinical Manifestations 308
Radiologic Features and Gross Pathology 308
Histopathology 308
Histologic Variants and Grading 309
Differential Diagnosis 310
Ancillary Diagnostic Studies 312
Genetics 312
Treatment and Prognosis 312
Smooth Muscle Tumors, Including Leiomyoma, Leiomyosarcoma, and EBV-Associated Smooth Muscle Tumor 312
Introduction 312
Localization and Clinical Manifestations 313
Radiologic Features and Gross Pathology 313
Histopathology 313
Histologic Variants and Grading 313
Differential Diagnosis 313
Ancillary Diagnostic Studies 313
Genetics 313
Treatment and Prognosis 313
Skeletal Muscle Tumors, Including Rhabdomyosarcoma Subtypes: Embryonal, Alveolar, and Spindle Cell/Sclerosing Subtypes 314
Definition and Synonyms 314
Localization and Clinical Manifestations 314
Radiologic Features and Gross Pathology 314
Histologic Variants and Grading 314
Differential Diagnosis 314
Ancillary Diagnostic Studies 314
Genetics 314
Treatment and Prognosis 316
Vascular Lesions, Including Malformations, Hemangiomas, Hemangioendothelioma, and Angiosarcoma 316
Definition and Synonyms 316
Localization and Clinical Manifestations 316
Radiologic Features and Gross Pathology 316
Histopathology 316
Histologic Variants and Grading 318
Differential Diagnosis 318
Ancillary Diagnostic Studies 318
Genetics 318
Treatment and Prognosis 318
Primitive Small Round Cell Tumors: Ewing Sarcoma, CIC-Sarcomas, and BCOR-Sarcomas 318
Definitions 318
Localization and Clinical Manifestations 318
15 Tumors of Peripheral Nerve 323
Abstract 323.e1
Keywords 323.e1
Introduction 323
Traumatic Neuroma 323
Definition and Synonyms 323
Pathogenesis 323
Clinical Manifestations and Localization 326
Radiologic Features and Gross Pathology 326
Histopathology 327
Differential Diagnosis 327
Ancillary Diagnostic/Prognostic Studies 329
Treatment and Prognosis 329
Schwannoma 329
Radiologic Features 330
Histologic Variants 331
Conventional Schwannoma 331
Incidence and Demographics. 331
Clinical Manifestations and Localization. 331
Gross Pathology. 331
Histopathology. 331
Ancillary Diagnostic/Prognostic Studies. 333
Differential Diagnosis. 333
Treatment and Prognosis. 335
Cellular Schwannoma 335
Demographics, Clinical Manifestations, and Localization. 335
Gross Pathology. 335
Histopathology. 335
Differential Diagnosis. 335
Treatment and Prognosis. 336
Plexiform Schwannoma 336
Incidence, Demographics, Clinical Manifestations, and Localization. 336
Gross Pathology. 337
Histopathology. 337
Differential Diagnosis. 338
Treatment and Prognosis. 338
Epithelioid Schwannoma 339
Malignant Transformation of Conventional, Cellular, and Plexiform Schwannoma 339
Melanotic Schwannoma 339
Incidence, Demographics, Clinical Manifestations, and Localization. 339
Gross Pathology. 339
Histopathology and Genetics. 339
Differential Diagnosis. 340
Neurofibroma 340
Definition and Genetics 340
Radiologic Features 341
Histologic Variants 341
Cutaneous Neurofibroma, Localized and Diffuse 342
Clinical Manifestations and Localization. 342
Gross Pathology. 342
Histopathology. 342
Ancillary Diagnostic/Prognostic Studies. 343
Treatment and Prognosis. 343
Differential Diagnosis. 343
Localized Intraneural Neurofibromas 343
Definition. 343
Incidence and Demographics. 344
Clinical Manifestations, Radiologic Features, and Localization. 344
Gross Pathology. 344
Histopathology. 344
Treatment and Prognosis. 344
Ancillary Diagnostic/Prognostic Studies. 344
Differential Diagnosis. 344
Atypical Changes in Neurofibromas 345
Plexiform Neurofibroma 345
Definition. 345
General Comments. 345
Gross Pathology. 345
Histopathology. 345
Ancillary Diagnostic/Prognostic Studies. 346
Treatment and Prognosis. 347
Differential Diagnosis. 349
Massive Soft Tissue Neurofibroma 349
Visceral Neurofibroma 349
Perineurioma 350
Intraneural Perineurioma 350
Definition 350
General Comments 350
Clinical Manifestations and Localization 350
Gross Pathology 350
Histopathology 350
Ancillary Diagnostic/Prognostic Studies 351
Differential Diagnosis 351
Treatment and Follow-Up 351
Soft Tissue Perineurioma 351
Definition 351
General Comments 351
Clinical Manifestations and Localization 351
Gross Pathology 352
Histopathology 352
Ancillary Diagnostic/Prognostic Studies 352
Differential Diagnosis 352
Treatment and Prognosis 352
Granular Cell Tumor 353
Definition and Synonyms 353
Clinical Manifestations and Localization 353
Gross Pathology 353
Histopathology 353
Ancillary Diagnostic/Prognostic Studies 353
Differential Diagnosis 355
Treatment and Prognosis 355
Miscellaneous Benign Neurogenic Tumors 355
Hybrid Nerve Sheath Tumors 355
Nerve Sheath Myxoma 356
Differential Diagnosis 356
Neurothekeoma 356
Ganglioneuroma 357
Definition and Synonyms 357
Introduction and Incidence 357
Demographics 358
Clinical Features 358
Radiologic Features 358
Gross Pathology 358
Microscopic Findings 358
Ancillary Diagnostic/Prognostic Studies 359
Genetics 359
Treatment and Prognosis 359
Primary Malignant Tumors of Nerve 359
Malignant Peripheral Nerve Sheath Tumor (MPNST) of Conventional Type 359
Definition and Synonyms 359
Introduction, Incidence, and Demographics 359
Manifestations and Localization 360
Radiologic Features 360
Gross Pathology 361
Histopathology 362
Ancillary Diagnostic/Prognostic Studies 364
Genetics 364
Differential Diagnosis 365
Treatment and Prognosis 365
MPNST Variants 366
Epithelioid MPNST 366
MPNST With Divergent Differentiation 368
MPNST With Perineurial Differentiation 368
MPNST Arising in Other Nervous System Tumors 369
Malignant Granular Cell Tumor 369
Definition 369
Demographics, Clinical Manifestations, and Localization 369
Gross Pathology 369
Histopathology 370
Ancillary Diagnostic/Prognostic Features 371
Differential Diagnosis 371
Treatment and Prognosis 371
Acknowledgments 371
References 371
16 Epithelial, Neuroendocrine, and Metastatic Lesions 375
Abstract 375.e1
Keywords 375.e1
Introduction 375
Metastatic Epithelial and Epithelioid Neoplasms 375
Introduction, Demographics, and Clinical Manifestations 375
Radiology and Gross Pathology Patterns of Metastasis 377
Histopathology and Ancillary Studies 378
Uncommon Metastases 380
Tumor-to-Tumor Metastasis in the Central Nervous System 385
Differential Diagnosis 385
Genetics, Treatment, and Prognosis 386
Paraganglioma 387
Introduction, Definition, and Synonyms 387
Incidence and Demographics 387
Clinical Manifestations 387
Radiologic Features and Gross Pathology 387
Histopathology, Differential Diagnosis, and Ancillary Studies 387
Treatment and Prognosis 389
Papillary Tumor of the Pineal Region 391
Definitions and Synonyms 391
Brief Historical Overview 391
Incidence and Demographics 391
Clinical Manifestations and Localization 391
Radiologic and Gross Features 391
Histopathology 391
Histologic Variants and Grading 391
Differential Diagnosis 391
Ancillary Diagnostic and Prognostic Studies 393
Genetics 393
Treatment and Prognosis 393
Cysts of the Central Nervous System 393
Epidermoid and Dermoid Cysts 395
Endodermal Cyst 397
Rathke Cleft Cyst 398
Colloid Cyst 398
Ancillary Stains for Endodermal, Rathke Cleft, and Colloid Cysts 400
Ependymal (Glioependymal) Cyst 400
Arachnoid Cyst 400
Choroid Plexus Cysts 401
Suggested Readings 403
References 403
17 Lymphomas and Histiocytic Tumors 405
Abstract 405.e1
Keywords 405.e1
Definition and Synonyms 405
Lymphomas 405
Brief Historical Overview 405
Incidence and Demographics 405
Clinical Manifestations and Localization 405
Radiologic Features and Gross Pathology 406
Histopathology and Immunohistochemistry 406
Histologic Variants 406
Immunodeficiency-Associated Lymphomas 406
Lymphomatoid Granulomatosis 410
Intravascular B Cell Lymphoma (Angiotropic Lymphoma) 410
Marginal Zone B Cell Lymphoma (MALT Lymphoma) of the Dura 410
Other Types of CNS Low-Grade B Cell Lymphoma 411
Plasmacytoma 411
Anaplastic Large-Cell Lymphoma 411
T Cell Lymphoma 411
Secondary CNS Involvement by Systemic Lymphomas and Leukemias 412
Myeloid Sarcoma 413
Differential Diagnosis 413
Other Brain Tumors Histologically Mimicking Lymphoma 413
“PCNSL Without PCNSL”: Sentinel and Corticosteroid-Treated Lesions 415
Demyelination 415
Encephalitis and Other Inflammatory Conditions 415
Vasculitis 415
Inflammatory Tumors and Pseudotumors 415
Ancillary Diagnostic Studies 415
Genetics 417
Treatment and Prognosis 417
Histiocytic Disorders 417
Brief Historical Overview 417
Incidence and Demographics 417
Clinical Manifestations and Localization 418
Radiologic Features and Gross Pathology 418
Histopathology and Immunohistochemistry 418
Non-Langerhans Cell Histiocytic Disorders 418
Suggested Readings 422
References 422
18 Germ Cell Tumors 423
Abstract 423.e1
Keywords 423.e1
Definition and Synonyms 423
Brief Historical Overview 423
Incidence and Demographics 424
Clinical Manifestations and Localization 424
Radiologic Features and Gross Pathology 425
Histopathology 426
Histologic Variants 426
Germinoma 426
Embryonal Carcinoma 427
Yolk Sac Tumor 427
Teratoma 428
General Comments 428
Mature Teratoma 428
Immature Teratoma 432
Teratoma With a Secondary Malignant Component 432
Congenital Teratomas 432
Choriocarcinoma 432
Mixed Germ Cell Tumors 434
Ancillary Diagnostic Studies 434
Placental Alkaline Phosphatase 434
Transcription Factors (Including OCT4 and SALL4) 434
CD30 437
C-Kit (CD117) 437
α-Fetoprotein 437
Glypican-3 437
Human Chorionic Gonadotropin and Human Placental Lactogen 437
LIN28A 437
Podoplanin and D2-40 437
Differential Diagnosis 438
Germinoma Versus Embryonal Carcinoma 438
Germinoma Versus Infiltrative Glioma or Lymphoma 439
Germinoma Versus Sarcoidosis or Infection 439
Embryonal Carcinoma Versus Yolk Sac Tumor 439
Embryonal Carcinoma or Yolk Sac Tumor Versus Metastatic Carcinoma 439
Choriocarcinoma Versus Syncytiotrophoblast-Containing Germ Cell Tumors 439
Genetics 439
Treatment and Prognosis 440
Germ Cell Tumors Metastatic to the Central Nervous System 440
Suggested Readings 440
References 441
19 Melanocytic Neoplasms of the Central Nervous System 443
Abstract 443.e1
Keywords 443.e1
Definitions and Synonyms 443
Brief Historical Overview 443
Meningeal Melanocytoma and Melanoma 443
Localization and Clinical Manifestations 443
Radiologic Features and Gross Pathology 443
Histopathology 444
Histologic Variants and Grading 446
Differential Diagnosis 446
Ancillary Diagnostic Studies 447
Genetics 447
Treatment and Prognosis 449
Meningeal Melanocytosis and Melanomatosis 449
Localization and Clinical Manifestations 449
Radiologic Features and Gross Pathology 449
Histopathology 449
Differential Diagnosis 450
Genetics 450
Treatment and Prognosis 450
Suggested Readings 450
References 450
20 Histologic Features of Pituitary Adenomas and Sellar Region Masses 453
Abstract 453.e1
Keywords 453.e1
Introduction 453
The Normal Pituitary 453
Embryologic Origin 453
Gross Anatomy of the Sellar Region 453
Histologic Features of Normal Anterior and Posterior Glands 454
Normal Histologic Variations Within the Gland 456
Specimen Handling of Sellar Region Tissues at Autopsy and as Surgical Specimens 457
Histochemical and Immunohistochemical Stains in the Workup of Permanent Sections From Sellar Region Masses 458
General Features of Pituitary Adenomas 459
Demographics 459
Clinical Symptoms 460
Neuroimaging Features 461
Molecular Genetics of Pituitary Adenomas 461
Frequency of Different Types of Adenomas 463
General Histologic Features of Pituitary Adenomas 463
Diagnosis of “Atypical” Pituitary Adenoma No Longer Utilized 463
Pituitary Carcinoma Is Diagnosed Only by Metastasis 466
Staging of Pituitary Adenomas 466
IHC Pattern for Specific Anterior Pituitary Hormones Versus Clinical Features and Serum Hormone Levels in Adenoma Patients 467
Clinically Nonfunctional/Clinically Silent Adenoma Is Not Identical to Hormone-Negative Adenoma or Null Cell Adenoma 468
Subtyping of Pituitary Adenomas May Predict Response to Therapies 468
Specific Subtypes of Pituitary Adenomas 469
Gonadotroph Adenoma 469
Hormone-Negative Adenoma (Formerly Null Cell Adenoma) 469
Growth Hormone Adenoma 470
Mixed GH/PRL Adenoma and Mammosomatotroph Cell Adenoma 473
Prolactin-Secreting Adenomas 473
Rare Prolactin-Secreting Adenoma Subtypes 473
Corticotroph Adenoma 473
TSH-Producing Adenoma and Pituitary Hyperplasia 475
Plurihormonal PIT1-Positive Adenoma 477
Ectopic Pituitary Adenoma 479
Pituitary Apoplexy 479
Mixed Pituitary Adenoma-Gangliocytoma 479
Hypophysitis 480
Rathke Cleft Cyst 482
Craniopharyngioma 485
Pituicytoma, Spindle Cell Oncocytoma, and Granular Cell Tumor of Neurohypophysis 486
Other Rare Sellar Region Masses 488
Acknowledgments 489
References 489
21 Therapy-Associated Neuropathology 493
Abstract 493.e1
Keywords 493.e1
Introduction 493
Radiation Necrosis and Other Forms of Radiation Injury 493
Definition and Synonyms 493
Brief Historical Overview 493
Incidence and Demographics 494
Localization and Clinical Manifestations 494
Radiologic Features and Gross Pathology 494
Histopathology 495
Histologic Variants and Grading 496
Differential Diagnosis 496
Ancillary Diagnostic Studies 496
Genetics 497
Treatment and Prognosis 497
Therapy-Induced Leukoencephalopathies and Vasculopathies 498
Definition and Synonyms 498
Brief Historical Overview 498
Incidence and Demographics 500
Localization and Clinical Manifestations 500
Radiologic Features and Gross Pathology 500
Histopathology 500
22 Familial Tumor Syndromes 505
Abstract 505.e1
Keywords 505.e1
Neurofibromatosis Type 1 505
Definition and Synonyms 505
Brief Historical Overview 508
Incidence and Demographics 508
Clinical Manifestations and Localization 508
Radiologic Features and Gross Pathology 509
Histopathology 509
Differential Diagnosis and Ancillary Diagnostic Studies 511
Genetics 511
Treatment and Prognosis 512
Neurofibromatosis Type 2 512
Definition and Synonyms 512
Brief Historical Overview 512
Incidence and Demographics 512
Clinical Manifestations and Localization 512
Radiologic Features and Gross Pathology 513
Histopathology 513
Differential Diagnosis 514
Ancillary Diagnostic Studies 514
Genetics 514
Treatment and Prognosis 515
Schwannomatosis 515
Definition and Synonyms 515
Brief Historical Overview 515
Incidence and Demographics 515
Clinical Manifestations and Localization 515
Radiologic Features and Gross Pathology 515
Histopathology 515
Differential Diagnosis 515
Ancillary Diagnostic Studies 516
Genetics 516
Treatment and Prognosis 516
Tuberous Sclerosis Complex 516
Definition and Synonyms 516
Brief Historical Overview 516
Incidence and Demographics 516
Clinical Manifestations and Localization 516
Radiologic Features and Gross Pathology 516
Histopathology 519
Differential Diagnosis 519
Ancillary Diagnostic Studies 519
Genetics 519
Treatment and Prognosis 519
Von Hippel-Lindau Disease 520
Definition and Synonyms 520
Brief Historical Overview 520
Incidence and Demographics 520
Clinical Manifestations and Localization 520
Radiologic Features and Gross Pathology 520
Histopathology 520
Histologic Variants and Grading 520
Differential Diagnosis 520
Ancillary Diagnostic Studies 523
Genetics 523
Treatment and Prognosis 524
Constitutional Mismatch Repair Deficiency Syndrome 524
Definition and Synonyms 524
Brief Historical Overview 524
Incidence and Demographics 524
Clinical Manifestations and Localization 524
Radiologic Features and Gross Pathology 524
Histopathology 524
Differential Diagnosis 524
Ancillary Diagnostic Studies 524
Genetics 524
Treatment and Prognosis 525
Lynch Syndrome 525
Definition and Synonyms 525
Brief Historical Overview 526
Incidence and Demographics 526
Clinical Manifestations and Localization 526
Radiologic Features and Gross Pathology 526
Histopathology 526
Differential Diagnosis 526
Ancillary Diagnostic Studies 526
Genetics 526
Treatment and Prognosis 526
Familial Adenomatous Polyposis Type 1 526
Definition and Synonyms 526
23 Infections and Inflammatory Disorders 547
Abstract 547.e1
Keywords 547.e1
Meningitis 548
Definition and Synonyms 548
Incidence and Demographics 548
Acute Bacterial Meningitis 548
Acute Viral Meningitis 548
Subacute/Chronic Tuberculous and Fungal Meningitis 549
Clinical Manifestations and Localization 549
Bacterial Meningitis 549
Viral Meningitis 550
Chronic Tuberculous and Fungal Meningitis 550
Radiologic Features and Gross Pathology 551
Histopathology 555
Acute Bacterial Meningitis 555
Subacute to Chronic Bacterial Meningitis 556
Tuberculous Meningitis 556
Syphilitic Meningitis 557
Viral Meningitis 557
Fungal Meningitis 558
Differential Diagnosis 559
Acute Bacterial Meningitis 559
Subacute to Chronic Bacterial Meningitis 559
Ancillary Studies 560
Viral Meningitis 560
Tuberculous Meningitis 560
Fungal Meningitis 560
Syphilitic Meningitis 560
Viral Encephalitis 560
Viral Encephalitis in Immunocompetent Hosts 560
Definition and Synonyms 560
Incidence and Demographics 561
Clinical Manifestations and Localization 561
Radiologic Features and Gross Pathology 561
Histopathology 562
Viral Encephalitis in Immunocompromised Hosts 563
West Nile Virus Encephalomyelitis 563
Progressive Multifocal Leukoencephalopathy 565
Subacute Sclerosing Panencephalitis 566
Progressive Rubella Encephalitis 566
Congenital Zika Virus Infection 566
Congenital Cytomegalovirus Encephalitis and Cytomegalovirus Encephalomyelitis 568
Varicella-Zoster Encephalomyelitis 568
Human Immunodeficiency Virus Leukoencephalopathy 568
Parasitic Encephalitis in Immunocompetent and Immunocompromised Hosts 568
Neurocysticercosis 568
Toxoplasmosis 568
CNS Malaria 569
Amebic Encephalitis 570
Trypanosomiasis 570
Differential Diagnosis 570
Focal Discrete Central Nervous System Infection 571
Definition and Synonyms 571
Pathogenesis, Incidence, and Demographics 571
Localized Parenchymal Bacterial Infections 571
Clinical Manifestations and Localization 571
Radiologic Features and Gross Pathology 571
Histopathology 571
Localized Tuberculous Infections 572
Localized Fungal Infections 572
Subdural and Epidural Infections 573
Focal Infections Mimicking Neoplasms and Vice Versa 574
Inflammatory Conditions of the Pachymeninges and Leptomeninges Mimicking Infections or Dural Neoplasms 575
Neurosarcoidosis 575
Definition and Synonyms 575
Incidence and Demographics 575
Clinical Manifestations and Localization 575
Radiologic Features and Gross Pathology 575
Histopathology 575
Differential Diagnosis 576
Central Nervous System Manifestations of Rheumatoid Arthritis 576
Definition and Synonyms 576
Incidence and Demographics 577
Clinical Manifestations and Localization 577
Radiologic Features and Gross Pathology 577
Histopathology 577
Differential Diagnosis 577
Central Nervous System Manifestations of Granulomatosis With Polyangiitis 577
Definition, Incidence, and Demographics 577
Clinical Manifestations and Localization 578
Radiologic Features and Gross Pathology 578
Histopathology 578
Differential Diagnosis 578
Acknowledgment 578
Suggested Readings 578
References 578
24 White Matter and Myelin Disorders 581
Abstract 581.e1
Keywords 581.e1
Introduction 581
Multiple Sclerosis 581
Definitions and Synonyms 581
Brief Historical Overview 581
Incidence and Demographics 581
Localization and Clinical Manifestations 583
Radiologic Features and Gross Pathology 583
Histopathology 584
Active Plaques 584
Inactive Plaques 584
Histologic Variants and Grading 587
Acute Multiple Sclerosis (Marburg Type) 587
Acute Inflammatory Demyelinative Mass Presenting as a Neoplasm (Tumefactive Multiple Sclerosis) 587
Balo Concentric Sclerosis 589
Differential Diagnosis 589
Ancillary Diagnostic Studies 591
Genetics 591
Treatment and Prognosis 591
Neuromyelitis Optica 591
Definitions and Synonyms 591
Brief Historical Overview 592
Incidence and Demographics 592
Clinical Manifestations and Localization 592
Radiologic Features and Gross Pathology 592
Histopathology 593
Histologic Variants and Grading 593
Differential Diagnosis 595
Ancillary Diagnostic Studies 595
Genetics 595
Treatment and Prognosis 595
Acute Disseminated Encephalomyelitis 595
Definitions and Synonyms 595
Brief Historical Overview 595
Incidence and Demographics 595
Clinical Manifestations and Localization 595
Radiologic Features and Gross Pathology 596
Histopathology 596
Histologic Variants and Grading 596
Differential Diagnosis 596
25 Pathology of Epilepsy 617
Abstract 617.e1
Keywords 617.e1
Introduction 617
Processing Epilepsy Surgery Specimens 617
Focal Cortical Dysplasia 618
Definition and Synonyms 618
Brief Historical Overview 618
Incidence and Demographics 618
Clinical Manifestations and Localization 618
Radiologic Features 618
Gross Pathology 619
Histopathology 619
Histologic Variants and Classification 623
Differential Diagnosis 624
Ancillary Diagnostic and Prognostic Studies 624
Genetics 625
Treatment 625
Hippocampal Sclerosis 625
Definition 625
Historical Overview 625
Incidence and Demographics 625
Clinical Manifestations and Localization 625
Radiologic Features 625
Gross Pathology 625
Histopathology 625
Differential Diagnosis 627
Genetics 627
Treatment and Prognosis 627
Infarct and Porencephaly 627
Definitions 627
Historical Overview 627
Incidence and Demographics 627
Clinical Manifestations and Localization 627
Radiologic Features 627
Gross Pathology 627
Histopathology 628
Differential Diagnosis 628
Treatment and Prognosis 628
Rasmussen Encephalitis 628
Definition 628
Historical Overview 628
Incidence and Demographics 628
Clinical Manifestations and Localizations 628
Radiologic Features 629
Gross Pathology 629
Histopathology 629
Differential Diagnosis 629
Ancillary Diagnostic and Prognostic Studies 629
Treatment and Prognosis 630
Other Pathologies 630
Suggested Readings 630
Focal Cortical Dysplasia 630
Hippocampal Sclerosis 630
Infarct Including Porencephalic Cyst 630
Rasmussen Encephalitis 630
References 631
26 Vascular and Ischemic Disorders 633
Abstract 633.e1
Keywords 633.e1
Introduction 633
Brief Historical Overview 633
Ischemic Cerebral Infarct 634
Definition and Synonyms 634
Incidence and Demographics 634
Clinical Manifestations and Localization 634
Radiologic Features and Gross Pathology 635
Histopathology 635
Differential Diagnosis 636
Ancillary Diagnostic Studies 636
Treatment and Prognosis 638
Hypertensive Cerebrovascular Disease 638
Definition and Synonyms 638
Incidence and Demographics 638
Clinical Manifestations and Localization 638
Radiology and Gross Pathology 638
Histopathology 639
Differential Diagnosis and Ancillary Diagnostic Studies 641
Genetics 641
Treatment and Prognosis 641
Cerebral Amyloid Angiopathy 641
Definitions and Synonyms 641
Incidence and Demographics 641
Clinical Manifestations and Localization 641
Radiologic Features and Gross Pathology 641
Histopathology 642
Differential Diagnosis 642
Ancillary Diagnostic Studies 643
Genetics 643
27 Biopsy Pathology of Neurodegenerative Disorders in Adults 659
Abstract 659.e1
Keywords 659.e1
Alzheimer Disease 661
Definition and Synonyms 661
Brief Historical Overview 661
Incidence and Demographics 661
Clinical Manifestations and Localization 661
Radiologic Features and Gross Pathology 661
Histopathology 661
Histologic Variants and Grading 666
Differential Diagnosis 666
Ancillary Diagnostic Studies 666
Genetics 666
Treatment and Prognosis 666
Dementia With Lewy Bodies and Idiopathic Parkinson Disease 666
Definition and Synonyms 666
Brief Historical Overview 667
Incidence and Demographics 667
Clinical Manifestations and Localization 667
Radiologic Features and Gross Pathology 667
Histopathology 667
Histologic Variants and Grading 669
Differential Diagnosis 669
Genetics 669
Treatment and Prognosis 669
Progressive Supranuclear Palsy 670
Corticobasal Degeneration 670
Multiple System Atrophy 671
Frontotemporal Lobar Degeneration and Pick Disease 672
Definition and Synonyms 672
Brief Historical Overview 672
Incidence and Demographics 672
Clinical Manifestations and Localization 673
Radiologic Features and Gross Pathology 673
Histopathology 673
Differential Diagnosis 673
Genetics 675
Human Prion Diseases (Transmissible Spongiform Encephalopathies) Including Creutzfeldt-Jakob Disease 675
Definitions, Synonyms, and Proposed Mechanisms of Pathogenesis 675
Brief Historical Overview 675
Incidence and Demographics 676
Clinical Manifestations and Localization 676
Radiologic Features and Gross Pathology 676
Histopathology and Precaution 676
Histologic Variants and Grading 677
Differential Diagnosis 677
Ancillary Diagnostic Studies 679
Genetics 679
Treatment and Prognosis 679
Suggested Readings 679
References 680
Index 681
A 681
B 682
C 683
D 685
E 687
F 688
G 690
H 692
I 693
J 694
K 694
L 694
M 694
N 698
O 700
P 700
R 704
S 704
T 706
U 706
V 706
W 708
X 708
Y 708
Z 708
IBC_Clinical Key ad IBC1