TUMORS OF THE CENTRAL NERVOUS SYSTEM

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


Table of Contents

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

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