Contents List of abbreviations xi Foreword xii ICD-O topographical coding 1 ICD-O morphological coding 1 CNS tumours 2 1 Introduction to CNS tumours 7 2 Gliomas, glioneuronal tumours, and neuronal tumours 15 Introduction 16 Adult-type diffuse gliomas Astrocytoma, IDH-mutant 19 Oligodendroglioma, IDH-mutant and 1p/19qcodeleted 28 Glioblastoma, IDH-wildtype 39 Paediatric-type diffuse low-grade gliomas Diffuse astrocytoma, MYB- or MYBL1-altered 56 Angiocentric glioma 59 Polymorphous low-grade neuroepithelial tumour of the young 62 Diffuse low-grade glioma, MAPK pathway–altered 65 Paediatric-type diffuse high-grade gliomas Diffuse midline glioma, H3 K27–altered 69 Diffuse hemispheric glioma, H3 G34–mutant 74 Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype 77 Infant-type hemispheric glioma 81 Circumscribed astrocytic gliomas Pilocytic astrocytoma 83 High-grade astrocytoma with piloid features 90 Pleomorphic xanthoastrocytoma 94 Subependymal giant cell astrocytoma 100 Chordoid glioma 104 Astroblastoma, MN1-altered 107 Glioneuronal and neuronal tumours Ganglioglioma 111 Gangliocytoma 116 Desmoplastic infantile ganglioglioma / desmoplastic infantile astrocytoma 119 Dysembryoplastic neuroepithelial tumour 123 Diffuse glioneuronal tumour with oligodendroglioma-like features and nuclear clusters 127 Papillary glioneuronal tumour 130 Rosette-forming glioneuronal tumour 133 Myxoid glioneuronal tumour 136 Diffuse leptomeningeal glioneuronal tumour 139 Multinodular and vacuolating neuronal tumour 143 Dysplastic cerebellar gangliocytoma (Lhermitte– Duclos disease) 146 Central neurocytoma 149 Extraventricular neurocytoma 153 Cerebellar liponeurocytoma 156 Ependymal tumours Introduction 159 Supratentorial ependymoma 161 Supratentorial ependymoma, ZFTA fusion–positive 164 Supratentorial ependymoma, YAP1 fusion–positive 167 Posterior fossa ependymoma 169 Posterior fossa group A (PFA) ependymoma 172 Posterior fossa group B (PFB) ependymoma 175 Spinal ependymoma 177 Spinal ependymoma, MYCN-amplified 180 Myxopapillary ependymoma 183 Subependymoma 186 3 Choroid plexus tumours 189 Choroid plexus papilloma 190 Atypical choroid plexus papilloma 193 Choroid plexus carcinoma 195 4 Embryonal tumours 199 Medulloblastoma Introduction 200 Medulloblastomas, molecularly defined Medulloblastoma, WNT-activated 203 Medulloblastoma, SHH-activated and TP53-wildtype 205 Medulloblastoma, SHH-activated and TP53-mutant 208 Medulloblastoma, non-WNT/non-SHH 211 Medulloblastomas, histologically defined Medulloblastoma, histologically defined 213 Other CNS embryonal tumours Introduction 220 Atypical teratoid/rhabdoid tumour 221 Cribriform neuroepithelial tumour 226 Embryonal tumour with multilayered rosettes 228 CNS neuroblastoma, FOXR2-activated 232 CNS tumour with BCOR internal tandem duplication 235 CNS embryonal tumour NEC/NOS 238 5 Pineal tumours 241 Introduction 242 Pineocytoma 243 Pineal parenchymal tumour of intermediate differentiation 246 Pineoblastoma 249 Papillary tumour of the pineal region 253 Desmoplastic myxoid tumour of the pineal region, SMARCB1-mutant 256 6 Cranial and paraspinal nerve tumours 259 Introduction 260 Schwannoma 261 Neurofibroma 265 Perineurioma 269 Hybrid nerve sheath tumours 271 Malignant melanotic nerve sheath tumour 273 Malignant peripheral nerve sheath tumour 275 Cauda equina neuroendocrine tumour (previously paraganglioma) 279 7 Meningioma 283 8 Mesenchymal, non-meningothelial tumours involving the CNS 299 Introduction 300 Soft tissue tumours Fibroblastic and myofibroblastic tumours Solitary fibrous tumour 301 Vascular tumours Haemangiomas and vascular malformations 306 Haemangioblastoma 310 Skeletal muscle tumours Rhabdomyosarcoma 314 Tumours of uncertain differentiation Intracranial mesenchymal tumour, FET::CREB fusion–positive 317 CIC-rearranged sarcoma 320 Primary intracranial sarcoma, DICER1-mutant 323 Ewing sarcoma 326 Chondro-osseous tumours Chondrogenic tumours Mesenchymal chondrosarcoma 330 Chondrosarcoma 332 Notochordal tumours Chordoma 335 9 Melanocytic tumours 339 Introduction 340 Diffuse meningeal melanocytic neoplasms Melanocytosis and melanomatosis 341 Circumscribed meningeal melanocytic neoplasms Melanocytoma and melanoma 344 10 Haematolymphoid tumours involving the CNS 349 Introduction 350 Lymphomas CNS lymphomas Primary diffuse large B-cell lymphoma of the CNS 351 Immunodeficiency-associated CNS lymphomas 356 Lymphomatoid granulomatosis 358 Intravascular large B-cell lymphoma 360 Miscellaneous rare lymphomas in the CNS MALT lymphoma of the dura 362 Other low-grade B-cell lymphomas of the CNS 364 Anaplastic large cell lymphoma (ALK+/ALK−) 366 T-cell and NK/T-cell lymphomas 368 Histiocytic tumours Erdheim–Chester disease 370 Rosai–Dorfman disease 372 Juvenile xanthogranuloma 374 Langerhans cell histiocytosis 376 Histiocytic sarcoma 379 11 Germ cell tumours 381 12 Tumours of the sellar region 391 Introduction 392 Adamantinomatous craniopharyngioma 393 Papillary craniopharyngioma 397 Pituicytoma, granular cell tumour of the sellar region, and spindle cell oncocytoma 401 Pituitary adenoma / pituitary neuroendocrine tumour 406 Pituitary blastoma 415 13 Metastases to the CNS 417 Metastases to the brain and spinal cord parenchyma 418 Metastases to the meninges 421 14 Genetic tumour syndromes involving the CNS 423 Introduction 424 Neurofibromatosis type 1 426 Neurofibromatosis type 2 429 Schwannomatosis 434 Von Hippel–Lindau syndrome 437 Tuberous sclerosis 441 Li–Fraumeni syndrome 446 Cowden syndrome 449 Constitutional mismatch repair deficiency syndrome 452 Familial adenomatous polyposis 1 456 Naevoid basal cell carcinoma syndrome 458 Rhabdoid tumour predisposition syndrome 460 Carney complex 462 DICER1 syndrome 464 Familial paraganglioma syndromes 467 Melanoma-astrocytoma syndrome 471 Familial retinoblastoma 473 BAP1 tumour predisposition syndrome 475 Fanconi anaemia 478 ELP1-medulloblastoma syndrome 481 Contributors 483 Declaration of interests 489 IARC/WHO Committee for ICD-O 491 Sources 493 References 501 Subject index 557 Previous volumes in the series 56