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WHO Classification of Tumours, Thoracic Tumours 5th Edition
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The WHO Classification of Tumours

Thoracic Tumours is the fifth volume in the 5th edition of the WHO series on the classification of human tumours. This series (also known as the WHO Blue Books) is regarded as the gold standard for the diagnosis of tumours 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 tumour 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

• Oncologists

• Respiratory physicians

• Thoracic radiologists

• Cancer researchers

• Surgeons

• Epidemiologists

• Cancer registrars

This volume

• Prepared by 217 authors and editors

• Contributors from around the world

• More than 1000 high-quality images

• More than 3500 references

List of abbreviations xi
Foreword xii
ICD-O topographical coding 1
ICD-O morphological coding 1
Tumours of the lung 2
Tumours of the pleura and pericardium 4
Tumours of the heart 5
Mesenchymal tumours of the thorax 6
Tumours of the thymus 7
Germ cell tumours of the mediastinum 8
Haematolymphoid tumours of the mediastinum 8
Ectopic tumours of thyroid and parathyroid origin 9
TNM staging of lung, pleural, and thymic tumours 10
Carcinomas of the lung 11
Pleural mesothelioma 13
Epithelial tumours of the thymus 14
TNM staging of tumours of soft tissues 15
Staging of Hodgkin and non-Hodgkin lymphomas 17
1 Tumours of the lung 19
Introduction 20
Small diagnostic samples 29
Epithelial tumours
Bronchial papillomas 37
Sclerosing pneumocytoma 41
Alveolar adenoma 44
Papillary adenoma 46
Bronchiolar adenoma /
ciliated muconodular papillary tumour 48
Mucinous cystadenoma 51
Mucous gland adenoma 53
Precursor glandular lesions
Atypical adenomatous hyperplasia 55
Adenocarcinoma in situ 57
Minimally invasive adenocarcinoma 60
Invasive non-mucinous adenocarcinoma 64
Invasive mucinous adenocarcinoma 75
Colloid adenocarcinoma 79
Fetal adenocarcinoma 81
Enteric-type adenocarcinoma 83
Squamous precursor lesions
Squamous dysplasia and carcinoma in situ 85
Squamous cell carcinomas
Squamous cell carcinoma 89
Lymphoepithelial carcinoma 94
Large cell carcinomas
Large cell carcinoma 97
Adenosquamous carcinoma
Adenosquamous carcinoma 100
Sarcomatoid carcinomas
Pleomorphic carcinoma 103
Pulmonary blastoma 106
Carcinosarcoma 109
Other epithelial tumours
NUT carcinoma of the lung (see Ch. 5)
Thoracic SMARCA4-deficient undifferentiated tumour 111
Salivary gland–type tumours
Pleomorphic adenoma 115
Adenoid cystic carcinoma 117
Epithelial-myoepithelial carcinoma 119
Mucoepidermoid carcinoma 121
Hyalinizing clear cell carcinoma 123
Myoepithelioma and myoepithelial carcinoma 125
Lung neuroendocrine neoplasms
Introduction 127
Precursor lesion
Diffuse idiopathic pulmonary neuroendocrine cell
hyperplasia 130
Neuroendocrine tumours
Carcinoid/neuroendocrine tumour 133
Neuroendocrine carcinomas
Small cell lung carcinoma 139
Large cell neuroendocrine carcinoma 144
Tumours of ectopic tissues
Melanoma 150
Meningioma 152
Mesenchymal tumours specific to the lung
Pulmonary hamartoma 154
Pulmonary chondroma 156
Diffuse pulmonary lymphangiomatosis 158
Pleuropulmonary blastoma 160
Pulmonary artery intimal sarcoma 164
Congenital peribronchial myofibroblastic tumour 166
Primary pulmonary myxoid sarcoma with
EWSR1-CREB1 fusion 168
PEComatous tumours
Lymphangioleiomyomatosis 170
PEComa 172
Haematolymphoid tumours
Introduction 174
MALT lymphoma 175
Diffuse large B-cell lymphoma 178
Lymphomatoid granulomatosis 181
Intravascular large B-cell lymphoma 184
Pulmonary Langerhans cell histiocytosis 186
Erdheim–Chester disease 189
2 Tumours of the pleura and pericardium 193
Introduction 194
Mesothelial tumours
Benign and preinvasive mesothelial tumours
Adenomatoid tumour 196
Well-differentiated papillary mesothelial tumour 198
Mesothelioma in situ 200
Localized mesothelioma 202
Diffuse mesothelioma 204
Haematolymphoid tumours
Introduction 220
Primary effusion lymphoma 221
Diffuse large B-cell lymphoma associated with
chronic inflammation 224
3 Tumours of the heart 227
Introduction 228
Benign tumours
Papillary fibroelastoma 230
Cardiac myxoma 233
Cardiac fibroma 237
Cardiac rhabdomyoma 239
Adult cellular rhabdomyoma 241
Cardiac lipoma and lipomatous hypertrophy of the
atrial septum 243
Lipomatous hamartoma of the atrioventricular valve 245
Hamartoma of mature cardiac myocytes 247
Mesenchymal cardiac hamartoma 249
Cardiac haemangiomas 251
Conduction system hamartoma 253
Cystic tumour of the atrioventricular node 256
Malignant tumours
Cardiac angiosarcoma 258
Cardiac leiomyosarcoma 260
Cardiac undifferentiated pleomorphic sarcoma 262
Other sarcomas that may involve the heart 265
Haematolymphoid tumours
Introduction 266
Cardiac diffuse large B-cell lymphoma 267
Cardiac fibrin-associated diffuse large B-cell
lymphoma 269
4 Mesenchymal tumours of the thorax 273
Introduction 274
Mesenchymal tumours
Adipocytic tumours
Lipoma 276
Thymolipoma 278
Liposarcoma 280
Fibroblastic and myofibroblastic tumours
Desmoid fibromatosis 282
Solitary fibrous tumour 284
Calcifying fibrous tumour 286
Inflammatory myofibroblastic tumour 288
Myxofibrosarcoma 290
Vascular tumours
Haemangioma 292
Lymphangioma 294
Epithelioid haemangioendothelioma 296
Angiosarcoma 299
Skeletal muscle tumours
Rhabdomyosarcoma 301
Peripheral nerve sheath and neural tumours
Paraganglioma 303
Granular cell tumour 305
Schwannoma 307
Malignant peripheral nerve sheath tumour 309
Peripheral neuroblastic tumours 311
Tumours of uncertain differentiation
Synovial sarcoma 314
Undifferentiated small round cell sarcomas 316
5 Tumours of the thymus 319
Epithelial tumours
Introduction 320
Type A thymoma (including atypical subtype) 326
Type AB thymoma 329
Type B1 thymoma 332
Type B2 thymoma 336
Type B3 thymoma 340
Micronodular thymoma with lymphoid stroma 345
Metaplastic thymoma 347
Lipofibroadenoma 349
Thymic carcinoma
Introduction 351
Squamous cell carcinoma 354
Basaloid carcinoma 359
Lymphoepithelial carcinoma 361
NUT carcinoma of the thorax 364
Clear cell carcinoma 368
Low-grade papillary adenocarcinoma 370
Mucoepidermoid carcinoma 372
Thymic carcinoma with adenoid cystic
carcinoma–like features 375
Enteric-type adenocarcinoma 377
Adenocarcinoma NOS 379
Adenosquamous carcinoma 381
Sarcomatoid carcinoma 383
Undifferentiated carcinoma 386
Thymic carcinoma NOS 388
Thymic neuroendocrine neoplasms
Neuroendocrine tumours 389
Carcinoid/neuroendocrine tumour 391
Neuroendocrine carcinomas
Small cell carcinoma 395
Large cell neuroendocrine carcinoma 397
6 Germ cell tumours of the mediastinum 399
Introduction 400
Seminoma 402
Embryonal carcinoma 405
Yolk sac tumour 407
Choriocarcinoma 412
Teratoma 414
Mixed germ cell tumours 417
Germ cell tumours with somatic-type solid
malignancy 419
Germ cell tumours with associated haematological
malignancy 422
7 Haematolymphoid tumours of the mediastinum 425
Introduction 426
Primary mediastinal large B-cell lymphoma 427
MALT lymphoma 430
T-lymphoblastic leukaemia/lymphoma 432
Classic Hodgkin lymphoma 435
Mediastinal grey zone lymphoma 438
Follicular dendritic cell sarcoma 441
Myeloid sarcoma 443
8 Ectopic tumours of thyroid and parathyroid origin 445
Ectopic thyroid tumours 446
Ectopic parathyroid tumours 448
9 Metastases 451
Metastasis to the lung 452
Metastasis to the pleura 459
Metastasis to the thymus and mediastinal lymph
nodes 463
Metastasis to the heart and pericardium 467
10 Genetic tumour syndromes involving the thorax 471
Introduction 472
Li–Fraumeni syndrome 474
BAP1 tumour predisposition syndrome 476
Carney complex 478
Contributors 481
Declaration of interests 488
IARC/WHO Committee for ICD-O 490
Sources 491
References 499
Subject index 555
Previous volumes in the series 565


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