by
PETER C. BURGER, M.D.
Professor of Pathology, Oncology, and Neurosurgery
The Johns Hopkins University
School of Medicine
Baltimore, Maryland
and
BERND W. SCHEITHAUER, M.D.
Consultant and Professor of Pathology
Mayo Clinic and Mayo Graduate School of Medicine
Rochester, Minnesota
1 Introduction and Classification of Central Nervous System Tumors
1.1 Introduction
1.2 Classification of Central Nervous System Tumors
1.2.1 Histologic Classification
2 Normal Embryology and Anatomy
2.1 Embryology
2.2 Normal Macroscopic Anatomy
2.3 Normal Microscopic Anatomy
3 Tumors of Neuroglia and Choroid Plexus Epithelium
3.1 Astrocytic Neoplasms
3.1.1 Fibrillary or "Diffuse" Astrocytic Tumors
3.1.1.1 Astrocytoma
3.1.1.2 Anaplastic Astrocytoma
3.1.1.3 Glioblastoma Multiforme
3.1.1.4 Gliosarcoma
3.1.1.5 Protoplasmic Astrocytoma
3.1.1.6 Granular Cell Astrocytic Neoplasms
3.1.1.7 Gliomatosis Cerebri
3.1.1.8 Meningeal Gliomatosis
3.1.2 Other Astrocytic Tumors
3.1.2.1 Pilocytic Astrocytoma
3.1.2.2 Pleomorphic Xanthoastrocytoma
3.1.2.3 Subependymal Giant Cell Astrocytoma (Tuberous Sclerosis)
3.1.2.4 Infantile Desmoplastic Astrocytoma
3.1.2.5 Gliofibroma
3.2 Oligodendroglial Neoplasms
3.2.1 Oligodendroglioma and Anaplastic/Malignant Oligodendroglioma
3.3 Ependymal Neoplasms
3.3.1 Ependymoma and Anaplastic/Malignant Ependymoma
3.3.2 Subependymoma
3.4 Choroid Plexus Neoplasms
3.4.1 Choroid Plexus Papilloma and Carcinoma
3.5 Gliomas of Mixed Composition
3.5.1 Mixed Gliomas
3.6 Other Gliomas
3.6.1 Astroblastoma
3.6.2 Granular Cell Tumor of the Infundibulum
4 Neuronal and Glio-Neuronal Tumors
4.1 Gangliocytoma and Ganglioglioma
4.2 Desmoplastic Infantile Ganglioglioma
4.3 Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease)
4.4 Central Neurocytoma
4.5 Dysembryoplastic Neuroepithelial Tumor
4.6 Other Glio-Neuronal Lesions
5 Embryonal Tumors
5.1 Primitive Neuroectodermal Tumor
5.2 Medulloepithelioma
5.3 Neuroblastoma and Ganglioneuroblastoma
5.4 Olfactory Neuroblastoma (Esthesioneuroblastoma)
5.5 Retinoblastoma
5.6 Melanotic Neuroectodermal Tumor of Infancy
5.7 Ependymoblastoma
5.8 Medulloblastoma
5.9 Medullomyoblastoma and Melanotic Medulloblastoma
5.10 Other Embryonal Tumors
6 Tumors of the Pineal Gland
6.1 Pineocytoma
6.2 Pineoblastoma
6.3 Mixed Pineocytoma/Pineoblastoma and Pineal Parenchymal Neoplasms of
Intermediate Differentiation
6.4 Pineal Cyst
6.5 Gliomas of the Pineal Gland
7 Tumors of Uncertain Origin
7.1 Hemangioblastoma
7.2 Primitive Polar Spongioblastoma
7.3 Atypical Teratoid/Rhabdoid Tumor
8 Germ Cell Tumors
9 Tumors of Meningothelial Cells
9.1 Meningioma
9.2 Atypical and Malignant Meningiomas
10 Tumors of Mesenchymal Tissue
10.1 Vascular Tumors and Tumor-Like Lesions
10.1.1 Vascular Malformations
10.1.1.1 Arteriovenous Malformation
10.1.1.2 Cavernous Angioma
10.1.1.3 Venous Malformation
10.1.1.4 Capillary Telangiectasis
10.1.1.5 Other Vascular Lesions
10.1.2 Vascular Neoplasms
10.1.2.1 Hemangiopericytoma
10.1.2.2 Hemangioendothelioma
10.1.2.3 Angiosarcoma
10.2 Nonvascular Mesenchymal Tumors and Tumor-Like Lesions
10.2.1 Benign Nonvascular Tumors
10.2.1.1 Lipoma
10.2.1.2 Lipoma of the Internal Auditory Canal
10.2.1.3 Chondroma and Osteochondroma
10.2.1.4 Fibroma and Fibromatosis
10.2.1.5 Other Benign Mesenchymal Lesions
10.2.2 Malignant Nonvascular Tumors
10.2.2.1 Chordoma
10.2.2.2 Chondrosarcoma
10.2.2.3 Mesenchymal Chondrosarcoma
10.2.2.4 Rhabdomyosarcoma
10.2.2.5 Fibrosarcoma
10.2.2.6 Malignant Fibrous Histiocytoma
10.2.2.7 Other Sarcomas
11 Tumors of Paraganglionic Tissue
11.1 Paraganglioma
12 Primary Tumors of Hematopoietic Tissue
12.1 Primary Central Nervous System Lymphoma
12.2 Plasmacytoma
12.3 Microgliomatosis
13 Tumors of the Peripheral Nerve Sheath
13.1 Schwannoma
13.2 Neurofibroma
13.3 Malignant Peripheral Nerve Sheath Tumor
13.4 Granular Cell Tumor
14 Melanocytic Tumors
14.1 Melanocytoma and Malignant Melanoma
15 Craniopharyngiomas
15.1 Adamantinomatous Craniopharyngioma
15.2 Papillary Craniopharyngioma
16 Benign Cystic Lesions
16.1 Colloid Cyst of the Third Ventricle
16.2 Rathke Cleft Cyst
16.3 Endodermal Cyst
16.4 Ependymal (Glioependymal) Cyst
16.5 Choroid Plexus Cysts
16.6 Epidermoid and Dermoid Cysts
16.7 Arachnoid Cyst
16.8 Pineal Cyst
16.9 Nerve Root Cyst
16.10 Synovial Cyst of Spine
16.11 Other Cysts
17 Tumor-Like Lesions of Maldevelopmental or Uncertain Origin
17.1 Hypothalamic Hamartoma
17.2 Nasal Cerebral Heterotopia ("Nasal Glioma")
17.3 Meningioangiomatosis
18 Dysgenetic Syndromes
18.1 Neurofibromatosis (von Recklinghausen Disease)
18.1.1 Neurofibromatosis 1
18.1.2 Neurofibromatosis 2
18.2 Other Dysgenetic Syndromes
18.2.1 Von Hippel-Lindau Syndrome
18.2.2 Tuberous Sclerosis
18.2.3 Neurocutaneous Melanosis
18.2.4 Sturge-Weber Disease
18.2.5 Mesencephalo-Oculo-Facial Angiomatosis (Wyburn-Mason Syndrome)
18.2.6 Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber
Disease)
18.2.7 Cowden Syndrome
19 Reactive and Inflammatory Masses Simulating Neoplasia
19.1 Demyelinating Disease
19.2 Cerebral Infarct
19.3 Radionecrosis
19.4 Langerhans Cell Histiocytosis
19.5 Xanthomatous Lesions
19.6 Progressive Multifocal Leukoencephalopathy
19.7 Sarcoidosis
19.8 Plasma Cell Granuloma
19.9 Extranodal Sinus Histiocytosis with Massive Lymphadenopathy
(Rosai-Dorfman Disease)
19.10 Castleman Disease
19.11 Syphilis (Gumma)
19.12 Collagen-Vascular Diseases
19.13 Amyloidoma
20 Metastatic and Secondary Neoplasms
20.1 Metastatic Carcinoma and Sarcoma
20.2 Leukemia
20.3 Secondary Malignant Lymphoma
20.4 Spinal Epidural Lymphoma
20.5 Intravascular Lymphoma
20.6 Lymphomatoid Granulomatosis
21 Diagnostic Methods in Neuro-Oncology
21.1 Frozen Section
21.2 Stereotactic Biopsy
21.3 Cytology
21.4 Immunohistochemistry
21.5 Special Stains
21.6 Cytologic Examination of Cerebrospinal Fluid
21.7 Proliferation Markers
21.8 DNA Cytofluorometry
21.9 Molecular Techniques
21.10 Neuroimaging
22 Appendices
22.1 A. Non-Neoplastic Lesions Potentially Misinterpreted as Neoplasms
22.2 B. CNS Tumors Potentially Overgraded as Aggressive or Anaplastic
Neoplasms
22.3 C. Suspect Diagnoses in Surgical Neuropathology
22.4 D. Frequently Cystic Lesions
22.5 E. Frequently Calcified Tumors
22.6 F. Intraventricular Tumors
22.7 G. Differential Diagnoses of Intraspinal Lesions by Anatomic
Location
22.8 H. Immunoreactivity of Selected CNS Tumors
22.9 I. Immunocytology of Primary Intracranial Germ Cell Tumors
23 Index