WHO CLASSIFICATION OF TUMOURS: CENTRAL NERVOUS SYSTEM TUMOURS

DESCRIPTION

The WHO Classificationof Tumours Central Nervous System Tumours is thesixth volume in the 5th edition of the WHO series on the classificationof human tumours. This series (also known as the WHO Blue Books) is regardedas the gold standard for the diagnosis of tumours and comprises a unique synthesis of histopathologicaldiagnosis with digital and molecular pathology.

These authoritativeand concise reference booksprovide indispensable internationalstandards for anyone involved in the care of patientswith cancer or in cancer research,underpinning individual patienttreatment as well as research into allaspects of cancer causation, prevention, therapy, and education.

What""s new in this edition?

- The 5th edition,guided by the WHO Classification of Tumours Editorial Board, will establisha single coherent cancer classification presented across a collection of individual volumes organized on thebasis of anatomical site(digestive system, breast, soft tissue and bone, etc.) and structured in a systematic manner, with each tumourtype listed within a taxonomic classification: site, category, family (class), type, and subtype. - In each volume, theentities are now listed from benignto malignant and are described under an updated set of headings, includinghistopathology, diagnosticmolecular pathology, staging,and easy-to-read essentialand desirable diagnostic criteria.

Who should readthis book?

- Pathologists

- Neuro-oncologists

- Neuroradiologists

- Medical oncologists

- Radiation oncologists

- Neurosurgeons

- Oncology nurses

- Cancer researchers

- Epidemiologists

- Cancer registrars

This volume

- Prepared by199 authors and editors

- Contributors fromaround the world

- More than 1100 high-quality images

- More than 3600 references

TABLE OF CONTENTS

List of abbreviations xi

Foreword xii

ICD-O topographical coding 1

ICD-O morphological coding 1

CNS tumours 2

1 Introduction toCNS tumours 7

2 Gliomas, glioneuronaltumours, and neuronal tumours15

Introduction 16

Adult-type diffusegliomas

Astrocytoma, IDH-mutant 19

Oligodendroglioma, IDH-mutant and1p/19q[1]codeleted 28

Glioblastoma, IDH-wildtype 39

Paediatric-type diffuse low-grade gliomas

Diffuse astrocytoma,MYB- or MYBL1-altered 56

Angiocentric glioma 59

Polymorphous low-gradeneuroepithelial tumour of

the young62

Diffuse low-gradeglioma, MAPK pathwayaltered65

Paediatric-type diffuse high-grade gliomas

Diffuse midlineglioma, H3 K27altered 69

Diffuse hemisphericglioma, H3 G34mutant 74

Diffuse paediatric-typehigh-grade glioma,

H3-wildtype and IDH-wildtype 77

Infant-type hemisphericglioma 81

Circumscribed astrocyticgliomas

Pilocytic astrocytoma83

High-grade astrocytoma with piloid features90

Pleomorphic xanthoastrocytoma94

Subependymal giantcell astrocytoma 100

Chordoid glioma 104

Astroblastoma, MN1-altered 107

Glioneuronal and neuronal tumours

Ganglioglioma 111

Gangliocytoma 116

Desmoplastic infantileganglioglioma /

desmoplastic infantileastrocytoma 119

Dysembryoplastic neuroepithelial tumour 123

Diffuse glioneuronaltumour with

oligodendroglioma-like features and nuclear

clusters 127

Papillary glioneuronaltumour 130

Rosette-forming glioneuronal tumour 133

Myxoid glioneuronaltumour 136

Diffuse leptomeningealglioneuronal tumour 139

Multinodular and vacuolatingneuronal tumour 143

Dysplastic cerebellargangliocytoma (Lhermitte

Duclos disease)146

Central neurocytoma 149

Extraventricular neurocytoma 153

Cerebellar liponeurocytoma156

Ependymal tumours

Introduction 159

Supratentorial ependymoma 161

Supratentorial ependymoma, ZFTA fusionpositive 164

Supratentorial ependymoma, YAP1 fusionpositive 167

Posterior fossa ependymoma 169

Posterior fossa group A (PFA) ependymoma 172

Posterior fossa group B (PFB) ependymoma 175

Spinal ependymoma177

Spinal ependymoma,MYCN-amplified 180

Myxopapillary ependymoma183

Subependymoma 186

3 Choroid plexustumours 189

Choroid plexuspapilloma 190

Atypical choroidplexus papilloma 193

Choroid plexuscarcinoma 195

4 Embryonal tumours199

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-SHH211

Medulloblastomas, histologically defined

Medulloblastoma, histologically defined 213

Other CNS embryonaltumours

Introduction 220

Atypical teratoid/rhabdoid tumour 221

Cribriform neuroepithelialtumour 226

Embryonal tumourwith multilayered rosettes 228

CNS neuroblastoma, FOXR2-activated 232

CNS tumour withBCOR internal tandem duplication 235

CNS embryonal tumourNEC/NOS 238

5 Pineal tumours241

Introduction 242

Pineocytoma 243

Pineal parenchymal tumour of intermediate

differentiation 246

Pineoblastoma 249

Papillary tumourof the pineal region 253

Desmoplastic myxoidtumour of the pineal region,

SMARCB1-mutant 256

6 Cranial and paraspinalnerve tumours 259

Introduction 260

Schwannoma 261

Neurofibroma 265

Perineurioma 269

Hybrid nervesheath tumours 271

Malignant melanoticnerve sheath tumour 273

Malignant peripheralnerve 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 tissuetumours

Fibroblastic and myofibroblastic tumours

Solitary fibroustumour 301

Vascular tumours

Haemangiomas and vascular malformations 306

Haemangioblastoma 310

Skeletal muscletumours

Rhabdomyosarcoma 314

Tumours ofuncertain differentiation

Intracranial mesenchymaltumour, FET::CREB

fusionpositive 317

CIC-rearranged sarcoma 320

Primary intracranialsarcoma, DICER1-mutant 323

Ewing sarcoma 326

Chondro-osseous tumours

Chondrogenic tumours

Mesenchymal chondrosarcoma330

Chondrosarcoma 332

Notochordal tumours

Chordoma 335

9 Melanocytic tumours339

Introduction 340

Diffuse meningealmelanocytic neoplasms

Melanocytosis and melanomatosis 341

Circumscribed meningealmelanocytic neoplasms

Melanocytoma and melanoma 344

10 Haematolymphoid tumours involving the CNS 349

Introduction 350

Lymphomas

CNS lymphomas

Primary diffuselarge B-cell lymphoma of theCNS 351

Immunodeficiency-associated CNS lymphomas 356

Lymphomatoid granulomatosis358

Intravascular large B-cell lymphoma 360

Miscellaneous rare lymphomas in the CNS

MALT lymphoma ofthe dura 362

Other low-gradeB-cell lymphomas of the CNS 364

Anaplastic largecell lymphoma(ALK+/ALK−) 366

T-cell and NK/T-cell lymphomas 368

Histiocytic tumours

ErdheimChester disease 370

RosaiDorfman disease372

Juvenile xanthogranuloma374

Langerhans cell histiocytosis 376

Histiocytic sarcoma 379

11 Germ celltumours 381

12 Tumours ofthe sellar region 391

Introduction 392

Adamantinomatous craniopharyngioma 393

Papillary craniopharyngioma397

Pituicytoma, granular cell tumour ofthe sellar

region, and spindlecell oncocytoma 401

Pituitary adenoma / pituitary neuroendocrine tumour406

Pituitary blastoma415

13 Metastases tothe CNS 417

Metastases tothe brain and spinal cord parenchyma418

Metastases tothe meninges 421

14 Genetic tumoursyndromes involving the CNS 423

Introduction 424

Neurofibromatosis type 1 426

Neurofibromatosis type 2 429

Schwannomatosis 434

Von HippelLindausyndrome 437

Tuberous sclerosis441

LiFraumeni syndrome446

Cowden syndrome449

Constitutional mismatchrepair deficiency syndrome 452

Familial adenomatouspolyposis 1 456

Naevoid basal cellcarcinoma syndrome 458

Rhabdoid tumourpredisposition syndrome 460

Carney complex462

DICER1 syndrome 464

Familial paragangliomasyndromes 467

Melanoma-astrocytoma syndrome 471

Familial retinoblastoma 473

BAP1 tumour predispositionsyndrome 475

Fanconi anaemia 478

ELP1-medulloblastoma syndrome 481

Contributors 483

Declaration ofinterests 489

IARC/WHO Committee for ICD-O 491

Sources 493

References 501

Subject index557

Previous volumesin the series 568