Keeping abreast of the major strides made in the field of neuropathology, Essentials of Diagnostic Surgical Neuropathology brings out its second edition with the aim of keeping the neuroscience community updated with the new developments in neuropathology.

This second edition comes close on the heels of the fifth edition of the WHO Classification of Central Nervous System Tumours (WHO CNS5) which was published in 2021. This new edition has retained the concise, point wise format of the earlier edition, making it a handy reference book. While the major changes in this edition are in neoplastic pathology, this book also provides updates in the pathology of non-neoplastic lesions that need surgical intervention.

The highlights of the neoplastic section are:

Description of new tumour types and subtypes included in the WHO CNS5
Grading of tumours as per WHO CNS5
Relevant immune profile and diagnostic molecular pathology for each tumour according to WHO CNS5. Essential and desirable diagnostic criteria, as defined by WHO CNS5, in a tabular form at the end
The salient features of the non-neoplastic section include:

New section on Therapy-related Neuropathology
Recent classifications of vascular malformations and epilepsy related lesions
New section on Infectious and Inflammatory Lesions Mimicking Neoplasms
New chapter on Surgical Pathology of Spinal Dysraphism and Other Neural Tube Defects
Another important feature includes the addition of a new chapter on techniques viz.
Smears in the Rapid Diagnosis of Central Nervous System Lesions
Challenges in the Interpretation of Stereotactic Biopsies



Copyright
Dedication
Contents
Foreword
Foreword
Preface
Acknowledgments
Executive Committee of the Neurological Society of India: 2021 22
Contributors
1 Tumors of the Nervous System
1.1 Introduction to the Fifth Edition of the World Health Organization Classification of Tumors of the Central Nervous System (WHO CNS5)
1.2 Adult-Type Diffuse Gliomas
1.2.1 Astrocytoma, IDH-mutant
1.2.2 Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
1.2.3 Glioblastoma, IDH-wildtype
1.3 Circumscribed Astrocytic Gliomas
1.3.1 Pilocytic Astrocytoma
1.3.2 High-Grade Astrocytoma with Piloid Features
1.3.3 Pleomorphic Xanthoastrocytoma
1.3.4 Subependymal Giant Cell Astrocytoma
1.3.5 Chordoid Glioma
1.3.6 Astroblastoma, MN1-Altered
1.4 Pediatric-type Diffuse Low-Grade Gliomas
1.4.1 Diffuse Astrocytoma, MYB- or MYBL1-altered
1.4.2 Angiocentric Glioma
1.4.3 Polymorphous Low-grade Neuroepithelial Tumor of the Young
1.4.4 Diffuse Low-grade Glioma, MAPK Pathway-Altered
1.5 Pediatric-type Diffuse High-grade Gliomas
1.5.1 Diffuse Midline Glioma (DMG), H3K27-altered
1.5.2 Diffuse Hemispheric Glioma, H3 G34-mutant
1.5.3 Diffuse Pediatric-type High-grade Glioma, H3-wildtype and IDH-wildtype
1.5.4 Infant-type Hemispheric Glioma
1.6 Glioneuronal and Neuronal Tumors
1.6.1 Dysembryoplastic Neuroepithelial Tumor
1.6.2 Gangliocytoma and Ganglioglioma
1.6.3 Dysplastic Cerebellar Gangliocytoma (Lhermitte Duclos Disease)
1.6.4 Desmoplastic Infantile Astrocytoma and Ganglioglioma
1.6.5 Papillary Glioneuronal Tumor
1.6.6 Rosette-Forming Glioneuronal Tumor
1.6.7 Diffuse Leptomeningeal Glioneuronal Tumor
1.6.8 Central Neurocytoma, Extraventricular Neurocytoma, and Cerebellar Liponeurocytoma
1.6.9 Diffuse Glioneuronal Tumors with Oligodendroglioma-like Features and Nuclear Clusters
1.6.10 Myxoid Glioneuronal Tumor
1.6.11 Multinodular and Vacuolating Neuronal Tumor
1.7 Ependymal Tumors
1.7.1 Supratentorial Ependymomas
1.7.1.1 Supratentorial Ependymoma
1.7.1.2 Supratentorial Ependymoma, ZFTA Fusion-positive
1.7.1.3 Supratentorial Ependymoma, YAP1 Fusion-positive
1.7.2 Posterior Fossa Ependymomas
1.7.2.1 Posterior Fossa Ependymoma
1.7.2.2 Posterior Fossa Group A (PFA) Ependymoma
1.7.2.3 Posterior fossa group B (PFB) ependymoma
1.7.3 Spinal Ependymomas
1.7.3.1 Spinal Ependymoma
1.7.3.2 Spinal Ependymoma, MYCN-amplified
1.7.4 Myxopapillary ependymomas
1.7.5 Subependymoma
1.8 Choroid Plexus Tumors
1.9 Embryonal Tumors
1.9.1 Medulloblastoma
1.9.1.1 Medulloblastoma, Histologically Defined
1.9.1.2 Medulloblastoma, Molecularly Defined
1.9.2 Other CNS Embryonal Tumors
1.9.2.1 Atypical Teratoid/Rhabdoid Tumor (AT/RT)
1.9.2.2 Cribriform Neuroepithelial Tumor (CRINET)
1.9.2.3 Embryonal Tumor with Multilayered Rosettes (ETMR)
1.9.2.4 CNS Neuroblastoma, FOXR2-activated
1.9.2.5 CNS Tumor with BCOR Internal Tandem Duplication
1.9.2.6 CNS Embryonal Tumor NEC/NOS
1.10 Pineal Tumors
1.10.1 Pineocytoma, Pineal Parenchymal Tumor of Intermediate Differentiation, and Pineoblastoma
1.10.2 Papillary Tumor of the Pineal Region
1.10.3 Desmoplastic Myxoid Tumor of the Pineal Region, SMARCB1-mutant
1.11 Meningioma
1.12 Mesenchymal Non-meningothelial Tumors Involving the CNS
1.12.1 Solitary Fibrous Tumor
1.12.2 Hemangiomas and Vascular Malformations
1.12.3 Hemangioblastoma
1.12.4 Rhabdomyosarcoma
1.12.5 Intracranial Mesenchymal Tumor, FET::CREB Fusion Positive
1.12.6 CIC-rearranged Sarcoma
1.12.7 Primary Intracranial Sarcoma, DICER1-mutant
1.12.8 Ewing Sarcoma
1.12.9 Mesenchymal Chondrosarcoma
1.12.10 Chondrosarcoma
1.12.11 Chordoma
1.13 Cranial and Paraspinal Nerve Tumors
1.13.1 Schwannoma
1.13.2 Neurofibroma
1.13.3 Perineurioma
1.13.4 Hybrid Nerve Sheath Tumors
1.13.5 Malignant Melanotic Nerve Sheath Tumor
1.13.6 Malignant Peripheral Nerve Sheath Tumor
1.13.7 Cauda Equina Neuroendocrine Tumor (Previously CNS Paraganglioma)
1.14 Melanocytic Tumors
1.14.1 Diffuse Meningeal Melanocytic Neoplasms: Meningeal Melanocytosis and Meningeal Melanomatosis
1.14.2 Circumscribed Meningeal Melanocytic Neoplasms: Melanocytoma and Melanoma
1.15 Hematolymphoid Tumors Involving the CNS
1.15.1 CNS Lymphomas
1.15.1.1 Primary Diffuse Large B-cell Lymphoma of the CNS
1.15.1.2 Immunodeficiency-associated CNS Lymphoma
1.15.1.3 Lymphomatoid Granulomatosis
1.15.1.4 Intravascular Large B-cell Lymphoma
1.15.2 Miscellaneous Rare Lymphomas in the CNS
1.15.2.1 Mucosa-Associated Lymphoid Tissue Lymphoma of the Dura
1.15.2.2 Other Low-grade B-cell Lymphomas of the CNS
1.15.2.3 Anaplastic Large Cell Lymphoma (ALK+/ALK )
1.15.2.4 T-cell and NK/T-cell Lymphomas
1.15.3 Histiocytic Tumors
1.15.3.1 Erdheim Chester Disease
1.15.3.2 Rosai Dorfman Disease
1.15.3.3 Juvenile Xanthogranuloma
1.15.3.4 Langerhans Cell Histiocytosis
1.15.3.5 Histiocytic Sarcoma
1.16 Germ Cell Tumors
1.16.1 Germinoma
1.16.2 Teratoma
1.16.2.1 Mature Teratoma
1.16.2.2 Immature Teratoma
1.16.2.3 Teratoma with Somatic-type Malignancy
1.16.3 Embryonal Carcinoma
1.16.4 Yolk Sac Tumor
1.16.5 Choriocarcinoma
1.16.6 Mixed Germ Cell Tumors
1.17 Tumors of the Sellar Region
1.17.1 Tumors of the Pituitary
1.17.2 Adamantinomatous Craniopharyngioma
1.17.3 Papillary Craniopharyngioma
1.17.4 Pituicyte Tumor Family
1.17.5 Pituitary Blastoma
1.18 Genetic Tumor Syndromes Involving the CNS
1.18.1 Neurofibromatosis Group
1.18.2 Tuberous Sclerosis Complex (Bourneville Disease/Bourneville-Pringle Syndrome/Epiloia)
1.18.3 Von Hippel Lindau Syndrome
1.18.4 Rhabdoid Tumor Predisposition Syndrome
1.18.5 Constitutional Mismatch Repair Deficiency Syndrome
1.18.6 Other Genetic Tumor Syndromes
1.19 Metastases to the CNS
1.19.1 Metastases to the Brain and Spinal Cord Parenchyma
1.19.2 Metastases to the Meninges
1.20 Tumors of the Spine
1.20.1 Introduction
1.20.2 Benign Tumors of the Vertebral Column
1.20.3 Malignant Tumors of the Vertebral Column
1.20.4 Extramedullary Spinal Cord Tumors
1.20.5 Intramedullary Spinal Cord Tumors
1.20.6 Tumor-like Lesions of the Spinal Cord
1.20.7 Congenital malformations of the Spinal Cord
1.21 Cysts of the Central Nervous System
1.22 Therapy-related Neuropathology
1.22.1 Radiation Necrosis (Radionecrosis, Radiation Necrosis, or Cerebral Radiation Damage)
2 Aneurysms and Vascular Malformations of the Nervous System
2.1 Intracranial Aneurysms
2.2 Vascular Malformations of the Brain
2.2.1 Arteriovenous Malformation
2.2.2 Cerebral Cavernous Malformation (CCM) or Cavernoma
2.2.3 Venous Angioma/Developmental Venous Anomaly (DVA)
2.2.4 Capillary Telangiectasia
2.2.5 Arteriovenous Fistula
2.2.6 Acquired Vascular Malformations
2.3 Vascular Malformations of the Spinal Cord
2.4 Syndromic Association with Vascular Malformations of CNS
3 Traumatic Brain Injury
3.1 Classification of TBI by Severity of Injury
3.2 Pathoanatomic Classification
3.3 Classification by Physical Mechanism
3.4 Classification by Pathophysiology
3.5 Effects of TBI
4 Surgical Pathology of Drug-Resistant Epilepsy
4.1 Introduction
4.2 Hippocampal Sclerosis
4.3 Malformations of Cortical Development
4.4 Focal Cortical Dysplasia
5 Infectious and Inflammatory Lesions Mimicking Neoplasms
5.1 Tuberculosis
5.2 Fungal Infections
5.3 Parasitic Infections
5.4 Neurosarcoidosis
5.5 Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)
5.6 IgG4-Related Disease
5.7 Tumefactive Demyelination
6 Surgical Pathology of Spinal Dysraphism and Other Neural Tube Defects
6.1 Embryology
6.2 Incidence
6.3 Etiopathogenesis
6.4 Open Neural Tube Defects
6.4.1 Anencephaly and Craniorachischisis Totalis
6.4.2 Myelomeningocele and Meningocele
6.5 Closed Neural Tube Defects
6.5.1 Defect of Gastrulation
6.5.2 Defect of Dysjunction
6.5.3 Defect of Secondary Neurulation and Postneurulation
6.6 Anterior Sacral Meningocele
7 Miscellaneous
7.1 Smears in the Rapid Diagnosis of Central Nervous System Lesions
7.2 Challenges in the Interpretation of Stereotactic Biopsies
Suggested Reading
Index