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Contents WHO classifi cation 10 Anaplastic ganglioglioma 141 Introduction: WHO classifi cation and grading of tumours Dysplastic cerebellar gangliocytoma of the central nervous system 12 ( Lhermitte–Duclos disease) 142 Desmoplastic infantile astrocytoma and ganglioglioma 144 1 Diffuse astrocytic and oligodendroglial tumours 15 Papillary glioneuronal tumour 147 Introduction 16 Rosette-forming glioneuronal tumour 150 Diffuse astrocytoma, IDH-mutant 18 Diffuse leptomeningeal glioneuronal tumour 152 Gemistocytic astrocytoma, IDH-mutant 22 Central neurocytoma 156 Diffuse astrocytoma, IDH-wildtype 23 Extraventricular neurocytoma 159 Diffuse astrocytoma, NOS 23 Cerebellar liponeurocytoma 161 Anaplastic astrocytoma, IDH-mutant 24 Paraganglioma 164 Anaplastic astrocytoma, IDH-wildtype 27 Anaplastic astrocytoma, NOS 27 7 Tumours of the pineal region 169 Glioblastoma, IDH-wildtype 28 Pineocytoma 170 Giant cell glioblastoma 46 Pineal parenchymal tumour of intermediate differentiation 173 Gliosarcoma 48 Pineoblastoma 176 Epithelioid glioblastoma 50 Papillary tumour of the pineal region 180 Glioblastoma, IDH-mutant 52 Glioblastoma, NOS 56 8 Embryonal tumours 183 Diffuse midline glioma, H3 K27M–mutant 57 Medulloblastoma 184 Oligodendroglioma, IDH-mutant and 1p/19q-codeleted 60 Medulloblastoma, NOS 186 Oligodendroglioma, NOS 69 Medulloblastomas, genetically defi ned 188 Anaplastic oligodendroglioma, IDH-mutant and Medulloblastoma, WNT-activated 188 1p/19q-codeleted 70 Medulloblastoma, SHH-activated and TP53-mutant 190 Anaplastic oligodendroglioma, NOS 74 Medulloblastoma, SHH-activated and TP53-wildtype 190 Oligoastrocytoma, NOS 75 Medulloblastoma, non-WNT/non-SHH 193 Anaplastic oligoastrocytoma, NOS 76 Medulloblastomas, histologically defi ned 194 Medulloblastoma, classic 194 2 Other astrocytic tumours 79 Desmoplastic/nodular medulloblastoma 195 Pilocytic astrocytoma 80 Medulloblastoma with extensive nodularity 198 Pilomyxoid astrocytoma 88 Large cell / anaplastic medulloblastoma 200 Subependymal giant cell astrocytoma 90 Embryonal tumour with multilayered rosettes, Pleomorphic xanthoastrocytoma 94 C19MC-altered 201 Anaplastic pleomorphic xanthoastrocytoma 98 Embryonal tumour with multilayered rosettes, NOS 205 Other CNS embryonal tumours 206 3 Ependymal tumours 101 Medulloepithelioma 207 Subependymoma 102 CNS neuroblastoma 207 Myxopapillary ependymoma 104 CNS ganglioneuroblastoma 207 Ependymoma 106 CNS embryonal tumour, NOS 208 Papillary ependymoma 111 Atypical teratoid/rhabdoid tumour 209 Clear cell ependymoma 111 CNS embryonal tumour with rhabdoid features 212 Tanycytic ependymoma 111 Ependymoma, RELA fusion–positive 112 9 Tumours of the cranial and paraspinal nerves 213 Anaplastic ependymoma 113 Schwannoma 214 Cellular schwannoma 216 4 Other gliomas 115 Plexiform schwannoma 217 Chordoid glioma of the third ventricle 116 Melanotic schwannoma 218 Angiocentric glioma 119 Neurofi broma 219 Astroblastoma 121 Atypical neurofi broma 220 Plexiform neurofi broma 220 5 Choroid plexus tumours 123 Perineurioma 222 Choroid plexus papilloma 124 Hybrid nerve sheath tumours 224 Atypical choroid plexus papilloma 126 Malignant peripheral nerve sheath tumour (MPNST) 226 Choroid plexus carcinoma 128 MPNST with divergent differentiation 227 Epithelioid MPNST 228 6 Neuronal and mixed neuronal–glial tumours 131 MPNST with perineurial differentiation 228 Dysembryoplastic neuroepithelial tumour 132 Gangliocytoma 136 10 Meningiomas 231 Ganglioglioma 138 Meningioma 232 Meningioma variants 237 Intravascular large B-cell lymphoma 276 Meningothelial meningioma 237 Miscellaneous rare lymphomas in the CNS 276 Fibrous meningioma 237 Low-grade B-cell lymphomas 276 Transitional meningioma 238 T-cell and NK/T-cell lymphomas 276 Psammomatous meningioma 238 Anaplastic large cell lymphoma (ALK+/ALK–) 277 Angiomatous meningioma 238 MALT lymphoma of the dura 277 Microcystic meningioma 239 Secretory meningioma 239 14 Histiocytic tumours 279 Lymphoplasmacyte-rich meningioma 240 Langerhans cell histiocytosis 280 Metaplastic meningioma 240 Erdheim–Chester disease 281 Chordoid meningioma 240 Rosai–Dorfman disease 282 Clear cell meningioma 241 Juvenile xanthogranuloma 282 Atypical meningioma 241 Histiocytic sarcoma 283 Papillary meningioma 242 Rhabdoid meningioma 243 15 Germ cell tumours 285 Anaplastic (malignant) meningioma 244 Germinoma 288 Embryonal carcinoma 289 11 Mesenchymal, non-meningothelial tumours 247 Yolk sac tumour 290 Solitary fi brous tumour / haemangiopericytoma 249 Choriocarcinoma 290 Haemangioblastoma 254 Teratoma 291 Haemangioma 258 Mature teratoma 291 Epithelioid haemangioendothelioma 258 Immature teratoma 291 Angiosarcoma 259 Teratoma with malignant transformation 291 Kaposi sarcoma 259 Mixed germ cell tumour 291 Ewing sarcoma / peripheral primitive neuroectodermal tumour 259 16 Familial tumour syndromes 293 Lipoma 260 Neurofi bromatosis type 1 294 Angiolipoma 260 Neurofi bromatosis type 2 297 Hibernoma 260 Schwannomatosis 301 Liposarcoma 260 Von Hippel–Lindau disease 304 Desmoid-type fi bromatosis 260 Tuberous sclerosis 306 Myofi broblastoma 260 Li–Fraumeni syndrome 310 Infl ammatory myofi broblastic tumour 261 Cowden syndrome 314 Benign fi brous histiocytoma 261 Turcot syndrome 317 Fibrosarcoma 261 Mismatch repair cancer syndrome 317 Undifferentiated pleomorphic sarcoma / malignant Familial adenomatous polyposis 318 fi brous histiocytoma 261 Naevoid basal cell carcinoma syndrome 319 Leiomyoma 262 Rhabdoid tumour predisposition syndrome 321 Leiomyosarcoma 262 Rhabdomyoma 262 17 Tumours of the sellar region 323 Rhabdomyosarcoma 262 Craniopharyngioma 324 Chondroma 262 Adamantinomatous craniopharyngioma 327 Chondrosarcoma 263 Papillary craniopharyngioma 328 Osteoma 264 Granular cell tumour of the sellar region 329 Osteochondroma 264 Pituicytoma 332 Osteosarcoma 264 Spindle cell oncocytoma 334 12 Melanocytic tumours 265 18 Metastatic tumours 337 Meningeal melanocytosis 267 Meningeal melanomatosis 267 Contributors 342 Meningeal melanocytoma 268 Declaration of interest statements 349 Meningeal melanoma 269 IARC/WHO Committee for ICD-O 350 Sources of fi gures and tables 351 13 Lymphomas 271 References 356 Diffuse large B-cell lymphoma of the CNS 272 Subject index 402 Corticoid-mitigated lymphoma 275 List of abbreviations 408 Sentinel lesions 275 Immunodefi ciency-associated CNS lymphomas 275 AIDS-related diffuse large B-cell lymphoma 275 EBV+ diffuse large B-cell lymphoma, NOS 276 Lymphomatoid granulomatosis 276.
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