Central Nervous System Tumors: Diagnostic Pathology

✍ Scribed by César R. Lacruz (editor)

• Publisher: Springer
• Year: 2024
• Tongue: English
• Leaves: 464
• Edition: 1
• Category: Library

✦ Synopsis

Covering all aspects of CNS tumors pathology, this book provides complete information on tumors of the brain and its coverings, sellar region, pineal region, and cranial and paraspinal nerves according to 2021 WHO classification, 5th edition, including new entities, as well as benign cysts and nonneoplastic disorders that are potentially misinterpreted as neoplasms. Throughout the book, emphasis has been placed on histopathologic characteristics and differential diagnosis, which are summarized in tables to make the explanation of certain topics clearer.

This valuable text also includes the most recent clinical, pathological, histological, cytological, and molecular knowledge in the field, enabling pathologists to arrive at the correct diagnosis and prepare actionable, useful reports. Since pathology is one of the most visually oriented medical specialties, the myriad of annotated images throughout has been evaluated carefully and include neuroimaging studies, histology, cytology, and immunohistochemical stains that support a morphologic discussion in which differential diagnosis and potential pitfalls are emphasized.

Central Nervous System Tumors: Diagnostic Pathology provides a comprehensive guide of the current critical information needed to recognize, understand, and interpret this demanding field of surgical neuropathology. It is designed with the needs of practicing surgical pathologists, pathologists-in training, and clinicians in mind.

✦ Table of Contents

Preface
Acknowledgments
Contents
Contributors
1: Classification of the CNS Tumors
1.1 Brief Historical Overview
1.2 2021 WHO Classification, 5th Edition
1.2.1 Main Changes in the 2021 5th Edition
Diagnosis
Integrated and Layered Diagnosis
NOS/NEC Diagnoses
Terminology
Grading
Arabic vs. Roman Numerals
Grading within Tumor Types
Term Anaplastic Omitted
Integrated Grading Criteria
Upgrading Entities
Mitotic Count
Novel Diagnostic Technologies
Newly Recognized Tumor Types
Specific Changes
Diffuse Gliomas
Circumscribed Astrocytic Gliomas
Ependymomas
Embryonal Tumors
1.2.2 Final Considerations
Suggested Reading
2: Clinical and Radiological Approach
2.1 Clinical Considerations for Pathologists
2.1.1 Age and Location
Adults
Children and Adolescents
2.1.2 Relevant Data of Clinical History
Previous Diagnoses or CNS Biopsies
Previous Therapy for CNS Tumors
2.1.3 Family History
2.2 Neuroimaging Considerations for Pathologists
2.2.1 Principal Neuroimaging Modalities
Basic Principles
Computed Tomography (CT)
Magnetic Resonance Imaging (MRI)
Positron Emission Tomography (18FDG-PET)
Thallium-201 Single-Photon Emission Computed Tomography (SPECT)
Digital Subtraction Angiography (DSA)
2.2.2 Neuroimaging Evaluation
Anatomic Location
Pattern of Growth
Signal Intensity
Functional MR Sequences
Pattern of Contrast Enhancement
Secondary Features
Multifocality
Summary
Suggested reading
3: Adult-Type Diffuse Gliomas
3.1 Introduction
3.2 Astrocytoma, IDH-Mutant
3.2.1 Definition and Epidemiology
3.2.2 Localization and Clinical Manifestations
3.2.3 Imaging and Gross Pathology
3.2.4 Molecular/Genetics
3.2.5 Histopathology and Grading
IDH-Mutant Astrocytoma, CNS WHO Grade 2
IDH-Mutant Astrocytoma, CNS WHO Grade 3 (Previously Anaplastic Astrocytoma)
IDH-Mutant Astrocytoma, CNS WHO Grade 4 (Previously IDH-Mutant Glioblastoma)
3.2.6 Cytology
3.2.7 Immunophenotype
3.2.8 Molecular/Cytogenetic Studies
3.2.9 Differential Diagnosis
3.2.10 Treatment and Prognosis
3.3 Oligodendroglioma, IDH-Mutant and 1p/19q-Codeleted
3.3.1 Definition and Epidemiology
3.3.2 Localization and Clinical Manifestations
3.3.3 Imaging and Gross Pathology
3.3.4 Molecular/Genetics
3.3.5 Histopathology and Grading
Oligodendroglioma, CNS WHO Grade 2 (Previously Classic Oligodendroglioma)
Oligodendroglioma, CNS WHO Grade 3 (Previously Anaplastic Oligodendroglioma)
3.3.6 Cytology
3.3.7 Immunophenotype
3.3.8 Molecular/Cytogenetic Studies
3.3.9 Differential Diagnosis
3.3.10 Treatment and Prognosis
3.4 Glioblastoma, IDH-Wildtype
3.4.1 Definition and Epidemiology
3.4.2 Localization and Clinical Manifestations
3.4.3 Imaging and Gross Pathology
3.4.4 Molecular/Genetics
3.4.5 Histopathology
3.4.6 Cytology
3.4.7 Immunophenotype
3.4.8 Glioblastoma Subtypes/Patterns
Giant Cell Glioblastoma
Gliosarcoma
Epithelioid Glioblastoma
Small Cell Glioblastoma
Granular Cell Glioblastoma
Oligodendroglioma-Like Glioblastoma
Other Patterns
3.4.9 Molecular/Cytogenetics Studies
3.4.10 Differential Diagnosis
3.4.11 Treatment and Prognosis
Suggested Reading
Astrocytoma, IDH-Mutant
Oligodendroglioma, IDH-Mutant and 1p/19q-Codeleted
Glioblastoma, IDH-Wildtype
4: Pediatric-Type Diffuse Gliomas
4.1 Introduction
4.2 Diffuse Astrocytoma, MYB- or MYBL1-Altered
4.2.1 Definition and Epidemiology
4.2.2 Localization and Clinical Manifestations
4.2.3 Imaging and Gross Pathology
4.2.4 Molecular/Genetics
4.2.5 Histopathology
4.2.6 Cytology
4.2.7 Immunophenotype
4.2.8 Differential Diagnosis
4.2.9 Treatment and Prognosis
4.3 Angiocentric Glioma
4.3.1 Definition and Epidemiology
4.3.2 Localization and Clinical Manifestations
4.3.3 Imaging and Gross Pathology
4.3.4 Molecular/Genetics
4.3.5 Histopathology
4.3.6 Cytology
4.3.7 Immunophenotype
4.3.8 Differential Diagnosis
4.3.9 Treatment and Prognosis
4.4 Polymorphous Low-Grade Neuroepithelial Tumor of the Young
4.4.1 Definition and Epidemiology
4.4.2 Localization and Clinical Manifestations
4.4.3 Imaging and Gross Pathology
4.4.4 Molecular/Genetics
4.4.5 Histopathology
4.4.6 Cytology
4.4.7 Immunophenotype
4.4.8 Differential Diagnosis
4.4.9 Treatment and Prognosis
4.5 Diffuse Low-Grade Glioma, MAPK Pathway-Altered
4.5.1 Definition and Epidemiology
4.5.2 Localization and Clinical Manifestations
4.5.3 Imaging and Gross Pathology
4.5.4 Molecular/Genetics
4.5.5 Histopathology
4.5.6 Immunophenotype
4.5.7 Differential Diagnosis
4.5.8 Treatment and Prognosis
4.6 Diffuse Midline Glioma, H3 K27-Altered
4.6.1 Definition and Epidemiology
4.6.2 Localization and Clinical Manifestations
4.6.3 Imaging and Gross Pathology
4.6.4 Molecular/Genetics
4.6.5 Histopathology
4.6.6 Cytology
4.6.7 Immunophenotype
4.6.8 Differential Diagnosis
4.6.9 Staging
4.6.10 Treatment and Prognosis
4.7 Diffuse Glioma, H3.3 G34-Mutant
4.7.1 Definition and Epidemiology
4.7.2 Localization and Clinical Manifestations
4.7.3 Imaging and Gross Pathology
4.7.4 Molecular/Genetics
4.7.5 Histopathology
4.7.6 Immunophenotype/Molecular Studies
4.7.7 Differential Diagnosis
4.7.8 Staging
4.7.9 Treatment and Prognosis
4.8 Diffuse Pediatric-Type High-Grade Glioma, H3-Wildtype and IDH-Wildtype
4.8.1 Definition and Epidemiology
4.8.2 Localization and Clinical Manifestations
4.8.3 Imaging and Gross Pathology
4.8.4 Molecular/Genetics
4.8.5 Histopathology
4.8.6 Immunophenotype
4.8.7 Treatment and Prognosis
4.9 Infant-Type Hemispheric Glioma
4.9.1 Definition and Epidemiology
4.9.2 Localization, Imaging, and Clinical Features
4.9.3 Molecular/Genetics
4.9.4 Microscopic Features and Differential Diagnosis
Suggested Reading
Pediatric-Type Diffuse Low-Grade Gliomas
Pediatric-Type Diffuse High-Grade Gliomas
5: Circumscribed Astrocytic Gliomas
5.1 Introduction
5.2 Pilocytic Astrocytoma
5.2.1 Definition and Epidemiology
5.2.2 Localization and Clinical Manifestations
5.2.3 Imaging and Gross Pathology
5.2.4 Molecular/Genetics
5.2.5 Histopathology
5.2.6 Cytology
5.2.7 Subtypes
Pilomyxoid Astrocytoma (PMA)
Pilocytic Astrocytoma with Histologic Features of Anaplasia
5.2.8 Immunophenotype/Molecular Studies
5.2.9 Differential Diagnosis
5.2.10 Staging
5.2.11 Treatment and Prognosis
5.3 High-Grade Astrocytoma with Piloid Features
5.3.1 Definition and Epidemiology
5.3.2 Localization and Clinical Manifestations
5.3.3 Imaging and Gross Pathology
5.3.4 Molecular/Genetics
5.3.5 Histopathology
5.3.6 Immunophenotype
5.3.7 Differential Diagnosis
5.3.8 Treatment and Prognosis
5.4 Pleomorphic Xanthoastrocytoma
5.4.1 Definition and Epidemiology
5.4.2 Localization and Clinical Manifestations
5.4.3 Imaging and Gross Pathology
5.4.4 Molecular/Genetics
5.4.5 Histopathology
5.4.6 Cytology
5.4.7 Immunophenotype
5.4.8 Differential Diagnosis
5.4.9 Staging
5.4.10 Treatment and Prognosis
5.5 Subependymal Giant Cell Astrocytoma
5.5.1 Definition and Epidemiology
5.5.2 Localization and Clinical Manifestations
5.5.3 Imaging and Gross Pathology
5.5.4 Molecular/Genetics
5.5.5 Histopathology
5.5.6 Cytology
5.5.7 Immunophenotype
5.5.8 Differential Diagnosis
5.5.9 Treatment and Prognosis
5.6 Chordoid Glioma
5.6.1 Definition and Epidemiology
5.6.2 Localization and Clinical Manifestations
5.6.3 Imaging and Gross Pathology
5.6.4 Molecular/Genetics
5.6.5 Histopathology
5.6.6 Cytology
5.6.7 Immunophenotype
5.6.8 Differential Diagnosis
5.6.9 Treatment and Prognosis
5.7 Astroblastoma, MN1-Altered
5.7.1 Definition and Epidemiology
5.7.2 Localization and Clinical Manifestations
5.7.3 Imaging and Gross Pathology
5.7.4 Molecular/Genetics
5.7.5 Histopathology
5.7.6 Cytology
5.7.7 Immunohistochemistry
5.7.8 Differential Diagnosis
5.7.9 Treatment and Prognosis
Suggesting Reading
Pilocytic Astrocytoma
High-Grade Astrocytoma with Piloid Features
Pleomorphic Xanthoastrocytoma
Subependymal Giant Cell Astrocytoma
Chordoid Glioma
Astroblastoma, MN1-Altered
6: Glioneuronal and Neuronal Tumors
6.1 Introduction
6.2 Ganglioglioma
6.2.1 Definition and Epidemiology
6.2.2 Localization and Clinical Manifestations
6.2.3 Imaging and Gross Pathology
6.2.4 Molecular/Genetics
6.2.5 Histopathology
6.2.6 Cytology
6.2.7 Immunophenotype
6.2.8 Differential Diagnosis
6.2.9 Treatment and Prognosis
6.3 Gangliocytoma
6.3.1 Definition and Epidemiology
6.3.2 Histopathology
6.3.3 Cytology
6.3.4 Immunophenotype
6.3.5 Differential Diagnosis
6.3.6 Treatment and Prognosis
6.4 Desmoplastic Infantile Ganglioglioma and Astrocytoma
6.4.1 Definition and Epidemiology
6.4.2 Localization and Clinical Manifestations
6.4.3 Imaging and Gross Pathology
6.4.4 Molecular/Genetics
6.4.5 Histopathology
6.4.6 Cytology
6.4.7 Immunophenotype
6.4.8 Differential Diagnosis
6.4.9 Treatment and Prognosis
6.5 Dysembryoplastic Neuroepithelial Tumor
6.5.1 Definition and Epidemiology
6.5.2 Localization and Clinical Manifestations
6.5.3 Imaging and Gross Pathology
6.5.4 Molecular/Genetics
6.5.5 Histopathology
6.5.6 Cytology
6.5.7 Immunophenotype
6.5.8 Differential Diagnosis
6.5.9 Treatment and Prognosis
6.6 Papillary Glioneuronal Tumor
6.6.1 Definition and Epidemiology
6.6.2 Localization and Clinical Manifestations
6.6.3 Imaging and Gross Pathology
6.6.4 Molecular/Genetics
6.6.5 Histopathology
6.6.6 Cytology
6.6.7 Immunophenotype
6.6.8 Differential Diagnosis
6.6.9 Treatment and Prognosis
6.7 Rosette-Forming Glioneuronal Tumor
6.7.1 Definition and Epidemiology
6.7.2 Localization and Clinical Manifestations
6.7.3 Imaging and Gross Pathology
6.7.4 Molecular/Genetics
6.7.5 Histopathology
6.7.6 Cytology
6.7.7 Immunophenotype
6.7.8 Differential Diagnosis
6.7.9 Treatment and Prognosis
6.8 Myxoid Glioneuronal Tumor
6.8.1 Definition and Epidemiology
6.8.2 Localization and Clinical Manifestations
6.8.3 Imaging and Gross Pathology
6.8.4 Molecular/Genetics
6.8.5 Histopathology
6.8.6 Cytology
6.8.7 Immunophenotype
6.8.8 Differential Diagnosis
6.8.9 Treatment and Prognosis
6.9 Diffuse Leptomeningeal Glioneuronal Tumor
6.9.1 Definition and Epidemiology
6.9.2 Localization and Clinical Features
6.9.3 Imaging and Gross Pathology
6.9.4 Molecular/Genetics
6.9.5 Histopathology
6.9.6 Cytology
6.9.7 Immunophenotype
6.9.8 Molecular/Cytogenetics Studies
6.9.9 Differential Diagnosis
6.9.10 Treatment and Prognosis
6.10 Multinodular and Vacuolating Neuronal Tumor
6.10.1 Definition and Epidemiology
6.10.2 Localization and Clinical Manifestations
6.10.3 Imaging and Gross Pathology
6.10.4 Molecular/Genetics
6.10.5 Histopathology
6.10.6 Immunophenotype
6.10.7 Differential Diagnosis
6.10.8 Treatment and Prognosis
6.11 Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease)
6.11.1 Definition and Epidemiology
6.11.2 Localization and Clinical Manifestations
6.11.3 Imaging and Gross Pathology
6.11.4 Molecular/Genetics
6.11.5 Histopathology
6.11.6 Cytology
6.11.7 Immunophenotype
6.11.8 Differential Diagnosis
6.11.9 Treatment and Prognosis
6.12 Central Neurocytoma
6.12.1 Definition and Epidemiology
6.12.2 Localization and Clinical Manifestations
6.12.3 Imaging and Gross Pathology
6.12.4 Molecular/Genetics
6.12.5 Histopathology
6.12.6 Cytology
6.12.7 Immunophenotype
6.12.8 Differential Diagnosis
6.12.9 Treatment and Prognosis
6.13 Extraventricular Neurocytoma
6.13.1 Definition and Epidemiology
6.13.2 Localization and Clinical Manifestations
6.13.3 Imaging and Gross Pathology
6.13.4 Molecular/Genetics
6.13.5 Histopathology
6.13.6 Immunophenotype
6.13.7 Differential Diagnosis
6.13.8 Treatment and Prognosis
6.14 Cerebellar Liponeurocytoma
6.14.1 Definition and Epidemiology
6.14.2 Localization and Clinical Manifestations
6.14.3 Imaging and Gross Pathology
6.14.4 Molecular/Genetics
6.14.5 Histopathology
6.14.6 Cytology
6.14.7 Immunophenotype
6.14.8 Differential Diagnosis
6.14.9 Treatment and Prognosis
Suggested Reading
Ganglioglioma and Gangliocytoma
Desmoplastic Infantile Ganglioglioma and Astrocytoma
Dysembryoplastic Neuroepithelial Tumor
Papillary Glioneuronal Tumor
Rosette-Forming Glioneuronal Tumor
Myxoid Glioneuronal Tumor
Diffuse Leptomeningeal Glioneuronal Tumor
Multinodular and Vacuolating Neuronal Tumor
Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease)
Central Neurocytoma
Extraventricular Neurocytoma
Cerebellar Liponeurocytoma
7: Ependymal Tumors
7.1 Introduction
7.2 Ependymoma
7.2.1 Definition
7.2.2 Localization and Clinical Manifestations
7.2.3 Imaging and Gross Pathology
7.2.4 Molecular/Genetics
Supratentorial ZFTA-Fused Ependymoma
Supratentorial YAP1-Fused Ependymoma
PFA Ependymoma
PFB Ependymoma
Spinal Ependymomas
MYCN-Amplified Spinal Ependymoma
7.2.5 Histopathology and Grading
Ependymoma, CNS WHO Grade 2 (Previously Classic Ependymoma)
Ependymoma, CNS WHO Grade 3 (Previously Anaplastic Ependymoma)
Histological Patterns
Other Patterns
7.2.6 Cytology
7.2.7 Immunophenotype
Molecular Groups and Surrogate IHC
7.2.8 Differential Diagnosis
7.2.9 Treatment and Prognosis
Molecular Subgroup Prognosis
7.3 Myxopapillary Ependymoma
7.3.1 Definition and Epidemiology
7.3.2 Localization and Clinical Manifestations
7.3.3 Imaging and Gross Pathology
7.3.4 Molecular/Genetics
7.3.5 Histopathology
7.3.6 Cytology
7.3.7 Immunophenotype
7.3.8 Differential Diagnosis
7.3.9 Staging
7.3.10 Treatment and Prognosis
7.4 Subependymoma
7.4.1 Definition and Epidemiology
7.4.2 Localization and Clinical Manifestations
7.4.3 Imaging and Gross Pathology
7.4.4 Molecular/Genetics
7.4.5 Histopathology
7.4.6 Cytology
7.4.7 Immunophenotype
7.4.8 Differential Diagnosis
7.4.9 Treatment and Prognosis
Suggested Reading
Ependymoma
Myxopapillary Ependymoma
Subependymoma
8: Choroid Plexus Tumors
8.1 Definition an Epidemiology
8.2 Localization and Clinical Manifestations
8.3 Imaging and Gross Pathology
8.4 Molecular/Genetics
8.4.1 Choroid Plexus Papilloma (CPP)
8.4.2 Atypical Choroid Plexus Papilloma (ACPP)
8.4.3 Choroid Plexus Carcinoma (CPC)
8.5 Histopathological Types and Grading
8.5.1 Choroid Plexus Papilloma, CNS WHO Grade 1
8.5.2 Atypical Choroid Plexus Papilloma, CNS WHO Grade 2
8.5.3 Choroid Plexus Carcinoma, CNS WHO Grade 3
8.6 Cytology
8.7 Immunohistochemistry
8.8 Molecular Studies
8.9 Differential Diagnosis
8.10 Treatment and Prognosis
Suggested Reading
9: Embryonal Tumors
9.1 Introduction
9.2 Medulloblastoma
9.2.1 Definition and Epidemiology
9.2.2 Localization and Clinical Manifestations
9.2.3 Imaging and Gross Pathology
9.2.4 Molecular/Genetics
9.2.5 Classification of Medulloblastoma
9.2.6 Medulloblastomas, Histologically Defined
Classic Medulloblastoma (~70%)
Desmoplastic/Nodular Medulloblastoma (~20%)
Medulloblastoma with Extensive Nodularity (~4%)
Large Cell/Anaplastic Medulloblastomas (~10%)
9.2.7 Cytology
9.2.8 Medulloblastomas, Molecularly Defined
9.2.9 Immunophenotype
9.2.10 Differential Diagnosis
9.2.11 Treatment and Prognosis
9.3 Atypical Teratoid/Rhabdoid Tumor
9.3.1 Definition and Epidemiology
9.3.2 Localization and Clinical Manifestations
9.3.3 Imaging and Gross Pathology
9.3.4 Molecular/Genetics
9.3.5 Histopathology
9.3.6 Cytology
9.3.7 Immunophenotype
9.3.8 Molecular Studies
9.3.9 Differential Diagnosis
9.3.10 Treatment and Prognosis
9.4 Embryonal Tumor with Multilayered Rosettes
9.4.1 Definition and Epidemiology
9.4.2 Localization and Clinical Manifestations
9.4.3 Imaging and Gross Pathology
9.4.4 Molecular/Genetics
9.4.5 Histopathology
Embryonal Tumor with Abundant Neuropil and True Rosettes (ETANTR)
Ependymoblastoma
Medulloepithelioma
9.4.6 Immunophenotype
9.4.7 Molecular Studies
9.4.8 Differential Diagnosis
9.4.9 Treatment and Prognosis
9.5 CNS Neuroblastoma, FOXR2-Activated
9.5.1 Definition and Epidemiology
9.5.2 Localization and Clinical Manifestations
9.5.3 Imaging and Gross Pathology
9.5.4 Molecular/Genetics
9.5.5 Histopathology
9.5.6 Cytology
9.5.7 Immunophenotype
9.5.8 Differential Diagnosis
9.6 CNS Tumor with BCOR Internal Tandem Duplication
9.6.1 Definition and Epidemiology
9.6.2 Localization and Clinical Manifestations
9.6.3 Molecular/Genetics
9.6.4 Histopathology
9.6.5 Immunophenotype
9.6.6 Differential Diagnosis
9.6.7 Grading and Prognosis
Suggested Reading
Medulloblastoma
Atypical Teratoid/Rhabdoid Tumor
Embryonal Tumor with Multilayered Rosettes
CNS Neuroblastoma, FOXR2-Activated
CNS Tumor with BCOR Internal Tandem Duplication
10: Pineal Tumors
10.1 Introduction
10.2 Pineocytoma
10.2.1 Definition and Epidemiology
10.2.2 Localization and Clinical Manifestations
10.2.3 Imaging and Gross Pathology
10.2.4 Molecular/Genetics
10.2.5 Histopathology
10.2.6 Cytology
10.2.7 Immunophenotype
10.2.8 Differential Diagnosis
10.2.9 Treatment and Prognosis
10.3 Pineal Parenchymal Tumor of Intermediate Differentiation
10.3.1 Definition and Epidemiology
10.3.2 Localization and Clinical Manifestations
10.3.3 Imaging Features and Gross Pathology
10.3.4 Molecular/Genetics
10.3.5 Histopathology and Grading
10.3.6 Cytology
10.3.7 Immunophenotype
10.3.8 Differential Diagnosis
10.3.9 Treatment and Prognosis
10.4 Pineoblastoma
10.4.1 Definition and Epidemiology
10.4.2 Localization and Clinical Manifestations
10.4.3 Imaging and Gross Pathology
10.4.4 Molecular/Genetics
10.4.5 Histopathology
10.4.6 Cytology
10.4.7 Immunophenotype
10.4.8 Differential Diagnosis
10.4.9 Treatment and Prognosis
10.5 Pineal Anlage Tumor
10.6 Papillary Tumor of the Pineal Region
10.6.1 Definition and Epidemiology
10.6.2 Localization and Clinical Manifestations
10.6.3 Imaging and Gross Pathology
10.6.4 Molecular/Genetics
10.6.5 Histopathology and Grading
10.6.6 Cytology
10.6.7 Immunophenotype
10.6.8 Differential Diagnosis
10.6.9 Treatment and Prognosis
10.7 Desmoplastic Myxoid Tumor of the Pineal Region, SMARCB1-Mutant
Suggested Reading
Pineal Parenchymal Tumors
Pineal Anlage Tumor
Papillary Tumor of the Pineal Region
Desmoplastic Myxoid Tumor, SMARCB1-Mutant
11: Cranial and Paraspinal Nerve Tumors
11.1 Introduction
11.2 Schwannoma
11.2.1 Definition and Epidemiology
11.2.2 Localization and Clinical Manifestations
11.2.3 Imaging and Gross Pathology
11.2.4 Molecular/Genetics
11.2.5 Histopathology
Conventional Schwannoma
Ancient Schwannoma
Cellular Schwannoma
11.2.6 Cytology
11.2.7 Immunophenotype
11.2.8 Differential Diagnosis
11.2.9 Treatment and Prognosis
11.3 Neurofibroma
11.3.1 Definition and Epidemiology
11.3.2 Localization and Clinical Manifestations
11.3.3 Imaging and Gross Pathology
11.3.4 Molecular/Genetics
11.3.5 Histopathology
Classic Neurofibroma
Ancient Neurofibroma
Cellular Neurofibroma
Plexiform Neurofibroma
Atypical Neurofibromatous Neoplasm of Uncertain Biological Potential (ANNUBP)
11.3.6 Cytology
11.3.7 Immunophenotype
11.3.8 Differential Diagnosis
11.3.9 Treatment and Prognosis
11.4 Malignant Melanotic Nerve Sheath Tumor
11.4.1 Definition and Epidemiology
11.4.2 Localization and Clinical Manifestations
11.4.3 Imaging and Gross Pathology
11.4.4 Molecular/Genetics
11.4.5 Histopathology
11.4.6 Cytology
11.4.7 Immunophenotype
11.4.8 Differential Diagnosis
11.4.9 Treatment and Prognosis
11.5 Malignant Peripheral Nerve Sheath Tumor
11.5.1 Definition and Epidemiology
11.5.2 Localization and Clinical Manifestations
11.5.3 Imaging Features and Gross Pathology
11.5.4 Molecular/Genetics
11.5.5 Histopathology and Grading
Grading
11.5.6 Cytology
11.5.7 Immunophenotype
11.5.8 In Situ Hybridization
11.5.9 Differential Diagnosis
11.5.10 Treatment and Prognosis
11.6 Cauda Equina Neuroendocrine Tumor
11.6.1 Definition and Epidemiology
11.6.2 Localization and Clinical Manifestations
11.6.3 Imaging and Gross Pathology
11.6.4 Molecular/Genetics
11.6.5 Histopathology
11.6.6 Cytology
11.6.7 Immunophenotype
11.6.8 Differential Diagnosis
11.6.9 Treatment and Prognosis
11.7 Ganglioneuroma
11.7.1 Definition and Epidemiology
11.7.2 Localization and Clinical Features
11.7.3 Imaging and Gross Pathology
11.7.4 Molecular/Genetics
11.7.5 Histopathology
11.7.6 Cytology
11.7.7 Immunophenotype
11.7.8 Differential Diagnosis
11.7.9 Treatment and Prognosis
Suggested Reading
Schwannoma
Neurofibroma
Malignant Melanotic Nerve Sheath Tumor
Malignant Peripheral Nerve Sheath Tumor
Cauda Equina Neuroendocrine Tumor
Ganglioneuroma
12: Meningioma
12.1 Definition and Epidemiology
12.2 Localization and Clinical Manifestations
12.3 Imaging Features and Gross Pathology
12.4 Etiology
12.5 Molecular/Genetics
12.6 Histopathology and Grading
12.6.1 Key Histopathological Features of Meningiomas
12.6.2 Meningothelial Meningioma
12.6.3 Fibrous Meningioma
12.6.4 Transitional Meningioma
12.6.5 Psammomatous Meningioma
12.6.6 Angiomatous Meningioma
12.6.7 Microcystic Meningioma
12.6.8 Secretory Meningioma
12.6.9 Lymphoplasmacyte-Rich Meningioma
12.6.10 Metaplastic Meningioma
12.6.11 Chordoid Meningioma
12.6.12 Clear Cell Meningioma
12.6.13 Papillary Meningioma
12.6.14 Rhabdoid Meningioma
12.6.15 Atypical Meningioma
12.6.16 Anaplastic (Malignant) Meningioma
12.7 Cytology
12.8 Immunophenotype and Proliferation Index
12.9 Prognostic Markers
12.10 Differential Diagnosis
12.11 Treatment and Prognosis
Suggested Reading
13: Mesenchymal, Non-meningothelial Tumors
13.1 Introduction
13.2 Solitary Fibrous Tumor
13.2.1 Definition and Epidemiology
13.2.2 Localization and Clinical Manifestations
13.2.3 Imaging Features and Gross Pathology
13.2.4 Molecular/Genetics
13.2.5 Histopathology and Grading
13.2.6 Cytology
13.2.7 Immunophenotype
13.2.8 Differential Diagnosis
13.2.9 Treatment and Prognosis
13.3 Hemangioblastoma
13.3.1 Definition and Epidemiology
13.3.2 Localization and Clinical Manifestations
13.3.3 Imaging Features and Gross Pathology
13.3.4 Molecular/Genetics
13.3.5 Histopathology
13.3.6 Cytology
13.3.7 Immunophenotype
13.3.8 Differential Diagnosis
13.3.9 Treatment and Prognosis
13.4 Lipoma
13.4.1 Definition and Epidemiology
13.4.2 Localization and Clinical Manifestations
13.4.3 Imaging Features and Gross Pathology
13.4.4 Histopathology
13.4.5 Differential Diagnosis
13.4.6 Treatment
13.5 Rhabdomyosarcoma
13.5.1 Definition and Epidemiology
13.5.2 Localization and Clinical Manifestations
13.5.3 Imaging Features and Gross Pathology
13.5.4 Molecular/Genetics
13.5.5 Histopathology
13.5.6 Cytology
13.5.7 Immunophenotype/Histochemistry
13.5.8 Differential Diagnosis
13.5.9 Treatment and Prognosis
13.6 Ewing Sarcoma
13.6.1 Definition and Epidemiology
13.6.2 Localization and Clinical Manifestations
13.6.3 Imaging Features and Gross Pathology
13.6.4 Molecular/Genetics
13.6.5 Histopathology
13.6.6 Cytology
13.6.7 Immunophenotype/Histochemistry
13.6.8 Molecular pathology
13.6.9 Differential Diagnosis
13.6.10 Treatment and Prognosis
13.7 CIC-Rearranged Sarcoma
13.7.1 Definition and Epidemiology
13.7.2 Localization and Clinical Manifestations
13.7.3 Imaging Features and Gross Pathology
13.7.4 Molecular/Genetics
13.7.5 Histopathology
13.7.6 Immunophenotype and Differential Diagnosis
13.7.7 Treatment and Prognosis
13.8 Intracranial Mesenchymal Tumor, FET::CREB Fusion-Positive
13.8.1 Definition and Epidemiology
13.8.2 Localization and Clinical Manifestations
13.8.3 Imaging Features and Gross Pathology
13.8.4 Molecular/Genetics
13.8.5 Histopathology
13.8.6 Immunophenotype
13.8.7 Differential Diagnosis
13.9 Primary Intracranial Sarcoma: DICER1-Mutant
13.9.1 Definition and Epidemiology
13.9.2 Localization and Clinical Manifestations
13.9.3 Molecular/Genetics
13.9.4 Histopathology
13.9.5 Immunophenotype
13.9.6 Differential Diagnosis
13.10 Chordoma
13.10.1 Definition and Epidemiology
13.10.2 Localization and Clinical Manifestations
13.10.3 Imaging Features and Gross Pathology
13.10.4 Molecular/Genetics
13.10.5 Histopathology
Conventional Chordoma
Dedifferentiated Chordoma
Poorly Differentiated Chordoma
13.10.6 Cytology
13.10.7 Immunophenotype
13.10.8 Differential Diagnosis
13.10.9 Treatment and Prognosis
13.11 Chondrosarcoma
13.11.1 Definition and Epidemiology
13.11.2 Localization and Clinical Manifestations
13.11.3 Molecular/Genetics
13.11.4 Imaging Features and Gross Pathology
13.11.5 Histopathology and Grading
13.11.6 Cytology
13.11.7 Immunophenotype
13.11.8 Differential Diagnosis
13.11.9 Treatment and Prognosis
13.12 Mesenchymal Chondrosarcoma
13.12.1 Definition and Epidemiology
13.12.2 Localization and Clinical Manifestations
13.12.3 Molecular/Genetics
13.12.4 Histopathology
13.12.5 Immunophenotype
13.12.6 Differential Diagnosis
13.12.7 Prognosis
13.13 Osteosarcoma
13.13.1 Definition and Epidemiology
13.13.2 Localization and Clinical Presentation
13.13.3 Molecular/Genetics
13.13.4 Histopathology
13.13.5 Cytology
13.13.6 Immunophenotype
13.13.7 Differential diagnosis
Suggested Reading
Solitary Fibrous Tumor
Hemangioblastoma
Lipoma
Rhabdomyosarcoma
Ewing Sarcoma
CIC-Rearranged Sarcoma
Intracranial Mesenchymal Tumor, FET::CREB Fusion-Positive
Primary Intracranial Sarcoma, DICER1-Mutant
Chordoma
Chondrosarcoma
Mesenchymal Chondrosarcoma
Osteosarcoma
14: Primary CNS Melanocytic Neoplasms
14.1 Introduction
14.2 Diffuse Meningeal Melanocytic Neoplasms: Melanocytosis and Melanomatosis
14.2.1 Definition and Epidemiology
14.2.2 Localization and Clinical Manifestations
14.2.3 Imaging Features and Gross Pathology
14.2.4 Molecular/Genetics
14.2.5 Histopathology
14.2.6 Cytology
14.2.7 Immunophenotype
14.2.8 Differential Diagnosis
14.2.9 Prognosis
14.3 Circumscribed Meningeal Melanocytic Neoplasms: Melanocytoma and Melanoma
14.3.1 Definition and Epidemiology
14.3.2 Localization and Clinical Manifestations
14.3.3 Imaging Features and Gross Pathology
14.3.4 Molecular/Genetics
14.3.5 Histopathology and Grading
Melanocytoma
Intermediate-Grade Melanocytic Neoplasm (IGMN)
Malignant Melanoma
14.3.6 Immunophenotype
14.3.7 Differential Diagnosis
14.3.8 Treatment and Prognosis
Suggested Reading
15: Primary CNS Lymphomas
15.1 Introduction
15.2 Primary Diffuse Large B-Cell Lymphoma of the CNS
15.2.1 Definition and Epidemiology
15.2.2 Localization and Clinical Manifestations
15.2.3 Imaging Features and Gross Pathology
15.2.4 Etiology
15.2.5 Molecular/Genetics
15.2.6 Histopathology
Corticoid-Mitigated Lymphoma
15.2.7 Cytology
15.2.8 Immunophenotype
15.2.9 Differential Diagnosis
15.2.10 Staging
15.2.11 Treatment and Prognosis
15.3 Immunodeficiency-Associated CNS Lymphomas
15.3.1 Definition and Epidemiology
15.3.2 Etiology
15.3.3 Imaging
15.3.4 Histopathology/Immunophenotype
15.4 Lymphomatoid Granulomatosis
15.4.1 Definition and Epidemiology
15.4.2 Localization and Clinical Features
15.4.3 Imaging Features and Gross Pathology
15.4.4 Histopathology and Grading
15.4.5 Diagnostic Molecular Pathology
15.4.6 Treatment and Prognosis
15.5 Intravascular Large B-Cell Lymphoma
15.5.1 Definition and Epidemiology
15.5.2 Localization and Clinical Manifestations
15.5.3 Imaging Features and Gross Pathology
15.5.4 Molecular/Genetics
15.5.5 Histopathology
15.5.6 Differential Diagnosis
15.5.7 Treatment and Prognosis
15.6 MALT Lymphoma of the Dura
15.6.1 Definition and Epidemiology
15.6.2 Localization and Clinical Manifestations
15.6.3 Imaging and Gross Pathology
15.6.4 Molecular/Genetics
15.6.5 Histopathology
15.6.6 Cytology
15.6.7 Immunophenotype/Molecular Pathology
15.6.8 Differential Diagnosis
15.6.9 Treatment and Prognosis
15.7 Other Low-Grade B-Cell Lymphomas of the CNS
15.7.1 Staging
15.8 Anaplastic Large-Cell Lymphoma (ALK+/ALK−)
15.8.1 Definition and Epidemiology
15.8.2 Localization and Clinical Manifestations
15.8.3 Molecular/Genetics
15.8.4 Histopathology
15.8.5 Immunophenotype/Molecular Diagnosis
15.8.6 Differential Diagnosis
15.8.7 Staging
15.9 T-Cell and NK/T-Cell Lymphomas
15.9.1 Primary CNS T-Cell Lymphoma
15.9.2 Primary CNS NK/T-Cell Lymphoma
Suggested Reading
Primary Diffuse Large B-Cell Lymphoma of the CNS
Immunodeficiency-Associated CNS Lymphomas
Lymphomatoid Granulomatosis
Intravascular Large B-Cell Lymphoma
MALT Lymphoma of the Dura
Other Low-Grade B-Cell Lymphomas of the CNS
Anaplastic Large-Cell Lymphoma (ALK+/ALK−)
T-Cell and NK/T-Cell Lymphomas
16: Histiocytic Tumors of the CNS
16.1 Introduction
16.2 Langerhans Cell Histiocytosis
16.2.1 Definition and Epidemiology
16.2.2 Localization and Clinical Manifestations
16.2.3 Imaging Features and Gross Pathology
16.2.4 Molecular/Genetics
16.2.5 Histopathology
16.2.6 Cytology
16.2.7 Immunophenotype
16.2.8 Electron Microscopy
16.2.9 Differential Diagnosis
16.2.10 Prognosis and Treatment
16.3 Rosai-Dorfman Disease
16.3.1 Definition and Epidemiology
16.3.2 Localization and Clinical Manifestations
16.3.3 Molecular/Genetics
16.3.4 Histopathology
16.3.5 Immunophenotype
16.3.6 Prognosis and Treatment
16.4 Erdheim-Chester Disease
16.4.1 Definition and Epidemiology
16.4.2 Localization and Clinical Manifestations
16.4.3 Molecular/Genetics
16.4.4 Histopathology
16.4.5 Immunophenotype
16.4.6 Prognosis and Treatment
16.5 Juvenile Xanthogranuloma
16.5.1 Definition and Epidemiology
16.5.2 Molecular/Genetics
16.5.3 Histopathology
16.5.4 Immunophenotype
16.5.5 Prognosis and Treatment
16.6 Histiocytic Sarcoma
16.6.1 Definition and Epidemiology
16.6.2 Imaging and Clinical Manifestations
16.6.3 Molecular/Genetics
16.6.4 Histopathology
16.6.5 Immunophenotype
16.6.6 Prognosis and Treatment
16.7 ALK-Positive Histiocytosis
Suggested Reading
Langerhans Cell Histiocytosis
Rosai-Dorfman Disease
Erdheim-Chester Disease
Juvenile Xanthogranuloma
Histiocytic Sarcoma
ALK-Positive Histiocytosis
17: Germ Cell Tumors of the CNS
17.1 Localization and Clinical Manifestations
17.2 Genetics
17.3 Germinoma
17.3.1 Histopathology
17.3.2 Cytology
17.3.3 Immunophenotype
17.3.4 Differential Diagnosis and Pitfalls
17.3.5 Treatment and Prognosis
17.4 Non-germinomatous Germ Cell Tumors
17.4.1 Histopathology and Immunophenotype
Embryonal Carcinoma
Yolk Sac Tumor
Choriocarcinoma
17.4.2 Cytology
17.4.3 Differential Diagnosis
17.4.4 Treatment and Prognosis
17.5 Teratomas
17.5.1 Histopathology
Mature Teratoma
Immature Teratoma
Teratoma with Somatic-Type Malignancy
Growing Teratoma Syndrome
17.5.2 Cytology
17.5.3 Immunophenotype
17.5.4 Differential Diagnosis
17.5.5 Treatment and Prognosis
17.6 Mixed Germ Cell Tumors
Suggested Reading
18: Tumors of the Sellar Region
18.1 Introduction
18.2 Adamantinomatous Craniopharyngioma
18.2.1 Definition and Epidemiology
18.2.2 Localization and Clinical Manifestations
18.2.3 Imaging Features and Gross Pathology
18.2.4 Genetics
18.2.5 Histopathology
18.2.6 Cytology
18.2.7 Immunophenotype
18.2.8 Differential Diagnosis
18.2.9 Treatment and Prognosis
18.3 Papillary Craniopharyngioma
18.3.1 Definition and Epidemiology
18.3.2 Localization and Clinical Manifestations
18.3.3 Imaging Features and Gross Pathology
18.3.4 Genetics
18.3.5 Histopathology
18.3.6 Cytology
18.3.7 Immunophenotype
18.3.8 Differential Diagnosis
18.3.9 Treatment and Prognosis
18.4 Pituitary Adenoma/Pituitary Neuroendocrine Tumor
18.4.1 Definition and Epidemiology
18.4.2 Localization and Clinical Manifestations
18.4.3 Imaging Features and Gross Pathology
18.4.4 Genetics
18.4.5 Histopathology
18.4.6 Histopathological Types/Subtypes
TPIT-Lineage Corticotroph Tumors
Densely Granulated Corticotroph Tumors
Sparsely Granulated Corticotroph Tumors
Crooke Cell Tumors
SF1-Lineage Gonadotroph Tumors
PIT1-Lineage Tumors
Somatotroph Tumors
Lactotroph Tumors
Thyrotroph Tumors
Mammosomatotroph Tumors
Mixed Somatotroph-Lactotroph Tumors
Acidophilic Stem Cell Tumors
Mature PIT1-Lineage Tumors (GH-Producing Plurihormonal Tumors)
Immature PIT1-Lineage Tumors
No Lineage Fidelity Tumors
Unclassified Plurihormonal Tumors
Null Cell Tumors
18.4.7 Cytology
18.4.8 Grading
18.4.9 Immunophenotype
18.4.10 Differential Diagnosis
18.4.11 Staging
18.4.12 Treatment and Prognosis
18.5 Pituitary Blastoma
18.6 Pituicytoma, Granular Cell Tumor of the Sellar Region, Spindle Cell Oncocytoma, and Sellar Ependymoma
18.6.1 Definition and Epidemiology
18.6.2 Related Terminology
18.6.3 Localization, Clinical Manifestations, and Imaging Features
18.6.4 Genetics
18.6.5 Histopathology
Pituicytoma
Granular Cell Tumor of the Sellar Region/Granular Pituicytoma
Spindle Cell Oncocytoma/Oncocytic Pituicytoma
Sellar Ependymoma/Ependymal Pituicytoma
18.6.6 Differential Diagnosis
18.6.7 Treatment and Prognosis
18.7 Other (Unusual) Tumors of the Sellar Region
Suggested Reading
Adamantinomatous and Papillary Craniopharyngiomas
Pituitary Adenoma/Pituitary Neuroendocrine Tumor
Pituitary Blastoma
Pituicytoma, Granular Cell Tumor of the Sellar Region, Spindle Cell Oncocytoma, and Sellar Ependymoma
Other Tumors of the Sellar Region
19: Metastases to the CNS and Its Coverings
19.1 Definition and Epidemiology
19.1.1 Frequency
19.1.2 Age and Prevalence
19.2 Localization
19.3 Clinical Features
19.4 Imaging and Gross Pathology
19.5 General Diagnostic Approach
19.6 Histopathology
19.7 Cytology
19.8 Immunophenotype
19.9 Diagnostic Molecular Pathology
19.10 Differential Diagnosis
19.11 Staging
19.12 Treatment and Prognosis
Suggested Reading
20: Tumor-Like Lesions
20.1 Introduction
20.2 Hypothalamic Hamartoma
20.3 Calcifying Pseudoneoplasm of the Neuraxis
20.4 Tumor-Like Demyelinating Lesion (Demyelinating Pseudotumor)
20.5 Radiation Necrosis (Radionecrosis)
20.6 Brain (Pyogenic) Abscess
20.7 Toxoplasmosis
20.8 Tuberculoma
20.9 Mycobacterial Spindle Cell Pseudotumor
20.10 Textiloma (Gossypiboma)
Suggested Reading
Hypothalamic Hamartoma
Calcifying Pseudoneoplasms of the Neuraxis
Tumor-Like Demyelinating Lesions
Radiation Necrosis
Brain Abscess
Toxoplasmosis
CNS Tuberculosis
Mycobacterial Pseudotumor
Textiloma
21: Benign Cystic Lesions of the Neuraxis
21.1 Introduction
21.2 Epidermoid Cyst
21.2.1 Microscopic Findings
21.2.2 Differential Diagnosis
21.2.3 Treatment and Prognosis
21.3 Dermoid Cyst
21.3.1 Microscopic Findings
21.3.2 Differential Diagnosis
21.3.3 Treatment and Prognosis
21.4 Colloid Cyst of the Third Ventricle
21.4.1 Microscopic Findings
21.4.2 Differential Diagnosis
21.4.3 Treatment and Prognosis
21.5 Rathke Cleft Cyst
21.5.1 Microscopic Findings
21.5.2 Differential Diagnosis
21.5.3 Treatment and Prognosis
21.6 Endodermal (Neurenteric) Cyst
21.6.1 Microscopic Findings
21.6.2 Differential Diagnosis
21.6.3 Treatment and Prognosis
21.7 Ependymal Cyst
21.7.1 Microscopic Findings
21.7.2 Differential Diagnosis
21.7.3 Treatment and Prognosis
21.8 Arachnoid Cyst
21.8.1 Microscopic Findings
21.8.2 Differential Diagnosis
21.8.3 Treatment and Prognosis
21.9 Pineal Cyst
21.9.1 Microscopic Features
21.9.2 Differential Diagnosis
21.9.3 Treatment and Prognosis
21.10 Simple Glial Cyst
Suggested Reading
Epidermoid and Dermoid Cysts
Colloid Cyst of the Third Ventricle
Rathke Cleft Cyst
Endodermal (Neurenteric) Cyst
Ependymal Cyst
Arachnoid Cyst
Pineal Cyst
Simple Glial Cyst
Index