CONTENTS

1. Overview and Classification ...... 1 2. Embryology and Normal Anatomy...... 5 Embryology ...... 5 Normal Anatomy...... 5 Skull ...... 5 Meninges ...... 6 Vasculature...... 8 Cerebrum...... 11 Cerebellum...... 24 Spine and Spinal Cord...... 27 Peripheral-Central Nervous System Junction ...... 29 3. Tumors of the Neuroglia and Choroid Plexus...... 33 Astrocytic Neoplasms...... 33 Diffusely Infiltrating Astrocytic Tumors ...... 33 Diffuse Astrocytoma...... 34 Anaplastic Astrocytoma...... 50 Glioblastoma ...... 55 Gliosarcoma...... 77 Granular Cell Astrocytoma ...... 80 Gliomatosis Cerebri...... 83 Meningeal Gliomatosis ...... 86 Protoplasmic Astrocytoma...... 87 Well-Circumscribed Astrocytic Neoplasms ...... 89 Pilocytic Astrocytoma ...... 89 Pleomorphic Xanthoastrocytoma...... 106 Subependymal Giant Cell Astrocytoma (Tuberous Sclerosis)...... 114 Desmoplastic Infantile Astrocytoma...... 118 Granular Cell Tumor of the Infundibulum...... 118 Pituicytoma ...... 120 Oligodendroglioma and Oligoastrocytoma...... 122 Ependymal Neoplasms...... 145 Ependymoma...... 145 Subependymoma...... 165 Mixed Gliomas...... 169 Other Gliomas ...... 170 Astroblastoma...... 170

ix Tumors of the Central Nervous System

Chordoid Glioma of the Third Ventricle...... 173 Angiocentric Glioma...... 175 Choroid Plexus Papilloma, Atypical Papilloma, and Carcinoma ...... 177 4. Neuronal and Glioneuronal Tumors...... 209 Gangliocytoma and Ganglioglioma...... 209 Desmoplastic Infantile Ganglioglioma ...... 218 Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease)...... 222 Neurocytic Neoplasms...... 225 Neurocytic Neoplasms of the Cerebral Hemispheres...... 225 Central Neurocytoma...... 225 Other Neurocytic Neoplasms of the Central Hemispheres (Extraventricular or Intraparenchymal Neurocytic Neoplasms)...... 230 Cerebellar Neurocytic Neoplasms ...... 233 Neurocytic Neoplasms of the Spinal Cord...... 234 Dysembryoplastic Neuroepithelial Tumor...... 235 Other Neuronal and Glioneuronal Tumors...... 241 Papillary Glioneuronal Tumor...... 241 Glioneuronal Tumor with Neuropil-Like (“Rosetted”) Islands...... 242 Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle...... 243 Malignant Neuronal and Glioneuronal Tumors...... 244 5. Embryonal Tumors...... 251 Introduction...... 251 Medulloblastoma...... 252 Medullomyoblastoma and Melanotic Medulloblastoma...... 267 Medulloepithelioma...... 269 Neuroblastoma and Ganglioneuroblastoma...... 270 Neuroblastic Tumor with Abundant Neuropil and True Rosettes...... 273 Ependymoblastoma...... 274 Tumors of the Trilateral Retinoblastoma Syndrome...... 275 Miscellaneous Small Cell Embryonal Tumors of the Brain and Spinal Cord ...... 275 Largely Undifferentiated Small Cell Embryonal Neoplasms...... 275 Small Cell Embryonal Tumors with Glial Differentiation...... 275 Small Cell Embryonal Tumors with Mixed Neuronal and Glial Differentiation . . . 276 Small Cell Embryonal Neoplasms with Mesenchymal Differentiation...... 276 Peripheral Primitive Neuroectodermal Tumor/Ewing’s Sarcoma ...... 276 Malignant Tumor Following Prophylactic Irradiation for Acute Lymphocytic Leukemia...... 276 Olfactory Neuroblastoma (Esthesioneuroblastoma)...... 277 Atypical Teratoid/Rhabdoid Tumor...... 279 Contents

Melanotic Neuroectodermal Tumor of Infancy...... 283 Polar Spongioblastoma...... 284 6. Tumors of the Pineal Region ...... 295 Pineal Parenchymal Tumors...... 295 Pineocytoma...... 295 Pineal Parenchymal Tumor of Intermediate Differentiation...... 298 Pineoblastoma...... 299 Papillary Tumor of the Pineal Region ...... 301 Pineal Cyst...... 303 Other Neoplasms and Non-Neoplastic Masses in the Pineal Region ...... 305 7. Hemangioblastoma...... 309 8. Germ Cell Tumors ...... 321 General Pathologic Features...... 321 Germinoma ...... 322 Teratoma...... 325 Yolk Sac Tumor...... 327 Embryonal Carcinoma...... 327 Choriocarcinoma...... 328 Treatment and Prognosis...... 328 9. Meningioma...... 331 10. Tumors and Tumor-Like Lesions of Mesenchymal Tissue...... 363 Vascular Malformations...... 363 Arteriovenous Malformation...... 363 Cavernous Angioma...... 365 Venous Malformation ...... 367 Capillary Telangiectasis...... 368 Vascular Malformations in Neurocutaneous Syndromes...... 369 Sturge-Weber Syndrome...... 369 Mesencephalo-Oculo-Facial Angiomatosis (Wyburn-Mason Syndrome)...... 370 Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Disease)...... 371 Vascular Neoplasms and Tumor-Like Lesions ...... 371 Papillary Endothelial Hyperplasia (Masson’s Vegetant Hemangioendothelioma). . 371 Capillary Hemangioma ...... 372 Epithelioid Hemangioendothelioma...... 372 Hemangiopericytoma...... 373 Angiosarcoma...... 378 Benign Nonvascular Mesenchymal Tumors and Tumor-Like Lesions...... 379 Solitary Fibrous Tumor...... 379 Lipoma (Lipomatous Hamartoma) ...... 380

xi Tumors of the Central Nervous System

Chondroma and Osteochondroma...... 382 Fibroma and Fibromatosis...... 383 Other Benign Mesenchymal Tumors and Tumor-Like Lesions...... 384 Malignant Nonvascular Mesenchymal Tumors and Tumor-Like Lesions...... 384 Chordoma ...... 384 Chondrosarcoma...... 390 Mesenchymal Chondrosarcoma ...... 390 Rhabdomyosarcoma...... 391 Fibrosarcoma ...... 392 Leiomyosarcoma ...... 394 Malignant Fibrous Histiocytoma...... 395 Other Sarcomas ...... 395 11. Paraganglioma ...... 409 12. Tumors of Lymphoid and Hematopoietic Tissue...... 411 Primary Tumors ...... 411 Primary Central Nervous System Lymphoma...... 411 Post-Transplant Lymphoproliferative Disease...... 420 Lymphomatoid Granulomatosis...... 420 Neurolymphomatosis...... 422 Plasmacytoma ...... 422 Amyloidoma...... 423 Microgliomatosis...... 424 Secondary Tumors ...... 424 Secondary Malignant Lymphomas ...... 424 Leukemia ...... 426 13. Tumors of Intracranial and Intraspinal Peripheral Nerves...... 431 Conventional Schwannoma...... 431 Cellular Schwannoma ...... 438 Melanotic Schwannoma...... 441 Neurofibroma...... 442 Malignant Peripheral Nerve Sheath Tumor...... 444 Lipoma and Rhabdomyoma (Hamartoma, Choristoma, Ectomesenchymoma) of Intracranial Peripheral Nerves...... 446 Other Tumors of Intracranial and Intraspinal Peripheral Nerves...... 447 14. Melanocytic Neoplasms...... 453 Primary Leptomeningeal Melanocytic Neoplasms ...... 453 Neurocutaneous Melanosis ...... 457 15. Craniopharyngiomas...... 461 Adamantinomatous Craniopharyngioma...... 461 Contents

Papillary Craniopharyngioma...... 465 16. Benign Cystic Lesions...... 471 Cysts Lined by Columnar Epithelium...... 471 Colloid Cyst of the Third Ventricle...... 471 Rathke Cleft Cyst...... 473 Endodermal (Enterogenous) Cyst ...... 476 Ependymal (Glioependymal) Cyst...... 478 Epidermoid and Dermoid Cysts...... 478 Arachnoid Cyst...... 481 Other Cysts...... 483 Choroid Plexus Cyst...... 483 Nerve Root (Tarlov) Cyst...... 483 Synovial Cyst of the Spine...... 483 Miscellaneous Cysts...... 484 17. Tumor-Like Lesions of Maldevelopmental or Uncertain Origin...... 491 Hypothalamic Hamartoma ...... 491 Nasal Cerebral Heterotopia (“Nasal Glioma”)...... 492 Meningioangiomatosis...... 493 Calcifying Pseudoneoplasm of the Neuraxis...... 495 Cortical Dysplasia...... 497 Other Malformative Lesions...... 499 18. Dysgenetic Diseases ...... 503 Neurofibromatosis 1 (Von Recklinghausen’s Disease)...... 503 Neurofibromatosis 2 (Central Neurofibromatosis) ...... 505 Von Hippel-Lindau Disease ...... 508 Tuberous Sclerosis Complex...... 510 Cowden’s Syndrome...... 513 Gorlin’s Syndrome...... 514 Turcot’s Syndrome...... 514 Other Dysgenetic Disorders...... 514 19. Reactive and Inflammatory Masses...... 519 Macrophage-Rich Lesions...... 519 Demyelinating Disease...... 519 Cerebral Infarct ...... 526 Progressive Multifocal Leukoencephalopathy...... 528 Langerhans Cell Histiocytosis ...... 531 Lymphoplasmacyte-Rich Mass Lesions...... 533 Plasma Cell Granuloma...... 533 Extranodal Sinus Histiocytosis with Massive Lymphadenopathy

xiii Tumors of the Central Nervous System

(Rosai-Dorfman Disease)...... 535 Castleman’s Disease...... 536 Sarcoidosis ...... 536 Xanthomatous Lesions...... 537 Xanthogranuloma and Xanthoma of the Choroid Plexus...... 537 Xanthomas Associated with Hyperlipidemia ...... 538 Xanthomas Associated with Systemic Disorders...... 538 Xanthomas in Weber-Christian Disease ...... 539 Xanthic Lesions Unassociated with Systemic Disease...... 539 Xanthogranulomatous Reaction to Benign Epithelial Cysts...... 539 Erdheim-Chester Disease...... 539 Collagen Vascular Diseases...... 539 Rheumatoid Disease...... 539 Wegener’s Granulomatosis...... 539 Systemic Lupus Erythematosus...... 539 Sjögren’s Syndrome...... 539 Radionecrosis...... 539 Pyogenic Abscess ...... 543 Textiloma (Gossypiboma) ...... 545 20. Metastatic and Secondary Neoplasms...... 553 Appendices...... 571 Index ...... 587