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WHO classification of tumours of the central nervous system 5th ed [Pehme köide]

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The WHO Classification of Tumours

Central Nervous System Tumours is the sixth volume in the 5th edition of the WHO series on the classification of human tumors. This series (also known as the WHO Blue Books) is regarded as the gold standard for the diagnosis of tumors and comprises a unique synthesis of histopathological diagnosis with digital and molecular pathology. These authoritative and concise reference books provide indispensable international standards for anyone involved in the care of patients with cancer or in cancer research, underpinning individual patient treatment as well as research into all aspects 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 establish a single coherent cancer classification presented across a collection of individual volumes organized on the basis of anatomical site (digestive system, breast, soft tissue and bone, etc.) and structured in a systematic manner, with each tumor type listed within a taxonomic classification: site, category, family (class), type, and subtype. In each volume, the entities are now listed from benign to malignant and are described under an updated set of headings, including histopathology, diagnostic molecular pathology, staging, and easy-to-read essential and desirable diagnostic criteria.

Who should read this book?
  • Pathologists
  • Neuro-oncologists
  • Neuroradiologists
  • Medical oncologists
  • Radiation oncologists
  • Neurosurgeons
  • Oncology nurses
  • Cancer researchers
  • Epidemiologists
  • Cancer registrars
This volume
  • Prepared by 199 authors and editors
  • Contributors from around the world
  • More than 1100 high-quality images
  • More than 3600 references
WHO Classification of Tumours Online
  • The content of this renowned classification series is now also available in a convenient digital format by purchasing a subscription directly from IARC here
  List of abbreviations
  xi  
Foreword   xii  
  ICD-0 topographical coding
  1 (1)
  ICD-0 morphological coding
  1 (6)
  CNS tumours
  2 (5)
  1 Introduction to CNS tumours
  7 (8)
  2 Gliomas, glioneuronal tumours, and neuronal tumours
  15 (174)
  Introduction
  16 (3)
  Adult-type diffuse gliomas Astrocytoma, IDH-mutant
  19 (37)
  Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
  28 (11)
  Glioblastoma, IDH-wildtype
  39 (17)
  Paediatric-type diffuse low-grade gliomas Diffuse astrocytoma, MYB- or MYBL1-altered
  56 (13)
  Angiocentric glioma
  59 (3)
  Polymorphous low-grade neuroepithelial tumour of the young
  62 (3)
  Diffuse low-grade glioma, MAPK pathway-altered
  65 (4)
  Paediatric-type diffuse high-grade gliomas Diffuse midline glioma, H3 K27-altered
  69 (14)
  Diffuse hemispheric glioma, H3 G34-mutant
  74 (3)
  Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype
  77 (4)
  Infant-type hemispheric glioma
  81 (2)
  Circumscribed astrocytic gliomas Pilocytic astrocytoma
  83 (28)
  High-grade astrocytoma with piloid features
  90 (4)
  Pleomorphic xanthoastrocytoma
  94 (6)
  Subependymal giant cell astrocytoma
  100 (4)
  Chordoid glioma
  104 (3)
  Astroblastoma, MN1-altered
  107 (4)
  Glioneuronal and neuronal tumours Ganglioglioma
  111 (48)
  Gangliocytoma
  116 (3)
  Desmoplastic infantile ganglioglioma /desmoplastic infantile astrocytoma
  119 (4)
  Dysembryoplastic neuroepithelial tumour
  123 (4)
  Diffuse glioneuronal tumour with oligodendroglioma-like features and nuclear clusters
  127 (3)
  Papillary glioneuronal tumour
  130 (3)
  Rosette-forming glioneuronal tumour
  133 (3)
  Myxoid glioneuronal tumour
  136 (3)
  Diffuse leptomeningeal glioneuronal tumour
  139 (4)
  Multinodular and vacuolating neuronal tumour
  143 (3)
  Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease)
  146 (3)
  Central neurocytoma
  149 (4)
  Extraventricular neurocytoma
  153 (3)
  Cerebellar liponeurocytoma
  156 (3)
  Ependymal tumours Introduction
  159 (30)
  Supratentorial ependymoma
  161 (3)
  Supratentorial ependymoma, ZFTA fusion-positive
  164 (3)
  Supratentorial ependymoma, YAPI fusion-positive
  167 (2)
  Posterior fossa ependymoma
  169 (3)
  Posterior fossa group A (PFA) ependymoma
  172 (3)
  Posterior fossa group B (PFB) ependymoma
  175 (2)
  Spinal ependymoma
  177 (3)
  Spinal ependymoma, MYCN-amplified
  180 (3)
  Myxopapillary ependymoma
  183 (3)
  Subependymoma
  186 (3)
  3 Choroid plexus tumours
  189 (10)
  Choroid plexus papilloma
  190 (3)
  Atypical choroid plexus papilloma
  193 (2)
  Choroid plexus carcinoma
  195 (4)
  4 Embryonal tumours
  199 (42)
  Medulloblastoma Introduction
  200 (3)
  Medulloblastomas, molecularly defined Medulloblastoma, WNT-activated
  203 (17)
  Medulloblastoma, SHH-activated and TP53-wildtype
  205 (3)
  Medulloblastoma, SHH-activated and TP53-mutant
  208 (3)
  Medulloblastoma, non-WNT/non-SHH
  211 (2)
  Medulloblastomas, histologically defined Medulloblastoma, histologically defined
  213 (7)
  Other CNS embryonal tumours Introduction
  220 (21)
  Atypical teratoid/rhabdoid tumour
  221 (5)
  Cribriform neuroepithelial tumour
  226 (2)
  Embryonal tumour with multilayered rosettes
  228 (4)
  CNS neuroblastoma, FOXR2-activated
  232 (3)
  CNS tumour with BCOR internal tandem duplication
  235 (3)
  CNS embryonal tumour NEC/NOS
  238 (3)
  5 Pineal tumours
  241 (18)
  Introduction
  242 (1)
  Pineocytoma
  243 (3)
  Pineal parenchymal tumour of intermediate differentiation
  246 (3)
  Pineoblastoma
  249 (4)
  Papillary tumour of the pineal region
  253 (3)
  Desmoplastic myxoid tumour of the pineal region, SMAflCB 7-mutant
  256 (3)
  6 Cranial and paraspinal nerve tumours
  259 (24)
  Introduction
  260 (1)
  Schwannoma
  261 (4)
  Neurofibroma
  265 (4)
  Perineurioma
  269 (2)
  Hybrid nerve sheath tumours
  271 (2)
  Malignant melanotic nerve sheath tumour
  273 (2)
  Malignant peripheral nerve sheath tumour
  275 (4)
  Cauda equina neuroendocrine tumour (previously paraganglioma)
  279 (4)
  7 Meningioma
  283 (16)
  8 Mesenchymal, non-meningothelial tumours involving the CNS
  299 (40)
  Introduction
  300 (1)
  Soft tissue tumours
 
  Fibroblastic and myofibroblastic tumours Solitary fibrous tumour
  301 (5)
  Vascular tumours Haemangiomas and vascular malformations
  306 (4)
  Haemangioblastoma
  310 (4)
  Skeletal muscle tumours Rhabdomyosarcoma
  314 (3)
  Tumours of uncertain differentiation Intracranial mesenchymal tumour, FET::CREB fusion-positive
  317 (3)
  CIC-rearranged sarcoma
  320 (3)
  Primary intracranial sarcoma, D/CER7-mutant
  323 (3)
  Ewing sarcoma
  326 (4)
  Chondro-osseous tumours Chondrogenic tumours Mesenchymal chondrosarcoma
  330 (9)
  Chondrosarcoma
  332 (3)
  Notochordal tumours Chordoma
  335 (4)
  9 Melanocytic tumours
  339 (10)
  Introduction
  340 (1)
  Diffuse meningeal melanocytic neoplasms Melanocytosis and melanomatosis
  341 (3)
  Circumscribed meningeal melanocytic neoplasms Melanocytoma and melanoma
  344 (5)
  10 Haematolymphoid tumours involving the CNS
  349 (32)
  Introduction
  350 (1)
  Lymphomas CNS lymphomas Primary diffuse large B-cell lymphoma of the CNS
  351 (11)
  Immunodeficiency-associated CNS lymphomas
  356 (2)
  Lymphomatoid granulomatosis
  358 (2)
  Intravascular large B-cell lymphoma
  360 (2)
  Miscellaneous rare lymphomas in the CNS MALT lymphoma of the dura
  362 (8)
  Other low-grade B-cell lymphomas of the CNS
  364 (2)
  Anaplastic large cell lymphoma (ALK+/ALK-)
  366 (2)
  T-cell and NK/T-cell lymphomas
  368 (2)
  Histiocytic tumours Erdheim-Chester disease
  370 (11)
  Rosai-Dorfman disease
  372 (2)
  Juvenile xanthogranuloma
  374 (2)
  Langerhans cell histiocytosis
  376 (3)
  Histiocytic sarcoma
  379 (2)
  11 Germ cell tumours
  381 (10)
  12 Tumours of the sellar region
  391 (26)
  Introduction
  392 (1)
  Adamantinomatous craniopharyngioma
  393 (4)
  Papillary craniopharyngioma
  397 (4)
  Pituicytoma, granular cell tumour of the sellar region, and spindle cell oncocytoma
  401 (5)
  Pituitary adenoma / pituitary neuroendocrine tumour
  406 (9)
  Pituitary blastoma
  415 (2)
  13 Metastases to the CNS
  417 (6)
  Metastases to the brain and spinal cord parenchyma
  418 (3)
  Metastases to the meninges
  421 (2)
  14 Genetic tumour syndromes involving the CNS
  423 (60)
  Introduction
  424 (2)
  Neurofibromatosis type 1
  426 (3)
  Neurofibromatosis type 2
  429 (5)
  Schwannomatosis
  434 (3)
  Von Hippel-Lindau syndrome
  437 (4)
  Tuberous sclerosis
  441 (5)
  Li-Fraumeni syndrome
  446 (3)
  Cowden syndrome
  449 (3)
  Constitutional mismatch repair deficiency syndrome
  452 (4)
  Familial adenomatous polyposis 1
  456 (2)
  Naevoid basal cell carcinoma syndrome
  458 (2)
  Rhabdoid tumour predisposition syndrome
  460 (2)
  Carney complex
  462 (2)
  DICER 1 syndrome
  464 (3)
  Familial paraganglioma syndromes
  467 (4)
  Melanoma-astrocytoma syndrome
  471 (2)
  Familial retinoblastoma
  473 (2)
  BAP1 tumour predisposition syndrome
  475 (3)
  Fanconi anaemia
  478 (3)
  ELP1-medulloblastoma syndrome
  481 (2)
Contributors   483 (6)
Declaration of interests   489 (2)
IARC/WHO Committee for ICD-O   491 (2)
Sources   493 (8)
References   501 (56)
Subject index   557 (11)
Previous volumes in the series   568