List of abbreviations x
Foreword xi
1 Introduction to tumours of the digestive system 13
General introduction 14
Classification of neuroendocrine neoplasms
of the digestive system 16
TNM staging of well-differentiated neuroendocrine tumours
of the gastrointestinal tract 20
2 Tumours of the oesophagus 23
WHO classification of tumours of the oesophagus 24
TNM staging of tumours of the oesophagus 25
Introduction 28
Benign epithelial tumours and precursors
Squamous papilloma 30
Barrett dysplasia 32
Squamous dysplasia 36
Malignant epithelial tumours
Adenocarcinoma of the oesophagus and
oesophagogastric junction NOS 38
Adenoid cystic carcinoma 44
Adenosquamous and mucoepidermoid carcinomas 46
Squamous cell carcinoma NOS 48
Undifferentiated carcinoma 54
Neuroendocrine neoplasms 56
3 Tumours of the stomach 59
WHO classification of tumours of the stomach 60
TNM staging of carcinomas of the stomach 61
Introduction 64
Gastritis and metaplasia: precursors of gastric neoplasms 65
Benign epithelial tumours and precursors
Fundic gland polyps 67
Hyperplastic polyps 69
Dysplasia 71
Intestinal-type adenoma 76
Foveolar-type adenoma 79
Pyloric gland adenoma 81
Oxyntic gland adenoma 83
Malignant epithelial tumours
Adenocarcinoma 85
Squamous cell carcinoma 96
Adenosquamous carcinoma 98
Undifferentiated carcinoma 100
Gastroblastoma 102
Neuroendocrine neoplasms 104
4 Tumours of the small intestine and ampulla 111
WHO classification of tumours of the small intestine
and ampulla 112
TNM staging of carcinomas of the small intestine 113
TNM staging of carcinomas of the ampulla of Vater 114
Introduction 116
Benign epithelial tumours and precursors
Non-ampullary adenoma 118
Ampullary adenoma 121
Malignant epithelial tumours
Non-ampullary adenocarcinoma 124
Ampullary adenocarcinoma 127
Neuroendocrine neoplasms 131
5 Tumours of the appendix 135
WHO classification of tumours of the appendix 136
TNM staging of adenocarcinomas of the appendix 137
Introduction 140
Epithelial tumours
Serrated lesions and polyps 141
Mucinous neoplasm 144
Adenocarcinoma 147
Goblet cell adenocarcinoma 149
Neuroendocrine neoplasms 152
6 Tumours of the colon and rectum 157
WHO classification of tumours of the colon and rectum 158
TNM staging of carcinomas of the colon and rectum 159
Introduction 162
Benign epithelial tumours and precursors
Serrated lesions and polyps 163
Conventional adenoma 170
Inflammatory bowel disease–associated dysplasia 174
Malignant epithelial tumours
Adenocarcinoma 177
Neuroendocrine neoplasms 188
7 Tumours of the anal canal 193
WHO classification of tumours of the anal canal 194
TNM staging of tumours of the anal canal and perianal skin 195
Introduction 196
Benign epithelial tumours and precursors
Inflammatory cloacogenic polyp 198
Condyloma 200
Squamous dysplasia (intraepithelial neoplasia) 202
Malignant epithelial tumours
Squamous cell carcinoma 205
Adenocarcinoma 208
Neuroendocrine neoplasms 212
8 Tumours of the liver and intrahepatic bile ducts 215
WHO classification of tumours of the liver and
intrahepatic bile ducts 216
TNM staging of tumours of the liver 217
TNM staging of tumours of the intrahepatic bile ducts 218
Introduction 220
Benign hepatocellular tumours
Focal nodular hyperplasia of the liver 221
Hepatocellular adenoma 224
Malignant hepatocellular tumours and precursors
Hepatocellular carcinoma 229
Hepatoblastoma 240
Benign biliary tumours and precursors
Bile duct adenoma 245
Biliary adenofibroma 248
Mucinous cystic neoplasm of the liver and biliary system 250
Biliary intraepithelial neoplasia (see Ch. 9)
Intraductal papillary neoplasm of the bile ducts
(see Ch. 9)
Malignant biliary tumours
Intrahepatic cholangiocarcinoma 254
Combined hepatocellular-cholangiocarcinoma and
undifferentiated primary liver carcinoma 260
Hepatic neuroendocrine neoplasms 263
9 Tumours of the gallbladder and extrahepatic bile ducts 265
WHO classification of tumours of the gallbladder and
extrahepatic bile ducts 266
TNM staging of tumours of the gallbladder 267
TNM staging of tumours of the perihilar bile ducts 268
TNM staging of tumours of the distal extrahepatic bile duct 269
Introduction 270
Benign epithelial tumours and precursors
Pyloric gland adenoma of the gallbladder 271
Biliary intraepithelial neoplasia 273
Intracholecystic papillary neoplasm 276
Intraductal papillary neoplasm of the bile ducts 279
Mucinous cystic neoplasm of the liver and biliary system
(see Ch. 8)
Malignant epithelial tumours
Carcinoma of the gallbladder 283
Carcinoma of the extrahepatic bile ducts 289
Neuroendocrine neoplasms of the
gallbladder and bile ducts 292
10 Tumours of the pancreas 295
WHO classification of tumours of the pancreas 296
TNM staging of carcinomas of the pancreas 297
Introduction 298
Benign epithelial tumours and precursors
Acinar cystic transformation 300
Serous neoplasms 303
Intraepithelial neoplasia 307
Intraductal papillary mucinous neoplasm 310
Intraductal oncocytic papillary neoplasm 315
Intraductal tubulopapillary neoplasm 317
Mucinous cystic neoplasm 319
Malignant epithelial tumours
Ductal adenocarcinoma 322
Acinar cell carcinoma 333
Pancreatoblastoma 337
Solid pseudopapillary neoplasm 340
Neuroendocrine neoplasms
Introduction 343
Non-functioning pancreatic neuroendocrine tumours 347
Functioning neuroendocrine tumours
Insulinoma 353
Gastrinoma 355
VIPoma 357
Glucagonoma 359
Somatostatinoma 361
ACTH-producing neuroendocrine tumour 363
Serotonin-producing neuroendocrine tumour 365
Neuroendocrine carcinoma 367
MiNENs 370
11 Haematolymphoid tumours of the digestive system 373
WHO classification of haematolymphoid tumours
of the digestive system 374
Introduction 376
Site-specific haematolymphoid tumours
Extranodal marginal zone lymphoma of mucosa-
associated lymphoid tissue (MALT lymphoma) 378
Duodenal-type follicular lymphoma 383
Enteropathy-associated T-cell lymphoma 386
Monomorphic epitheliotropic intestinal T-cell lymphoma 390
Intestinal T-cell lymphoma NOS 393
Indolent T-cell lymphoproliferative disorder of the GI tract 395
Hepatosplenic T-cell lymphoma 397
EBV+ inflammatory follicular dendritic cell sarcoma 399
Haematolymphoid tumours occurring with some frequency
in the digestive system
Diffuse large B-cell lymphoma 402
Follicular lymphoma 406
Mantle cell lymphoma 408
Burkitt lymphoma 411
Plasmablastic lymphoma 415
Posttransplant lymphoproliferative disorders 417
Extranodal NK/T-cell lymphoma 420
Systemic mastocytosis 423
Langerhans cell histiocytosis 426
Follicular dendritic cell sarcoma 428
Histiocytic sarcoma 430
12 Mesenchymal tumours of the digestive system 433
WHO classification of mesenchymal tumours
of the digestive system 434
TNM staging of gastrointestinal stromal tumours 435
Introduction 436
Gastrointestinal stromal tumour 439
Adipose tissue and (myo)fibroblastic tumours
Inflammatory myofibroblastic tumour 444
Desmoid fibromatosis 446
Solitary fibrous tumour 448
Lipoma 450
Inflammatory fibroid polyp 452
Plexiform fibromyxoma 454
Smooth muscle and skeletal muscle tumours
Leiomyoma 456
Leiomyosarcoma 458
Rhabdomyosarcoma 460
Vascular and perivascular tumours
Haemangioma 462
Epithelioid haemangioendothelioma 466
Kaposi sarcoma 468
Angiosarcoma 471
Glomus tumour 473
Lymphangioma and lymphangiomatosis 475
Neural tumours
Schwannoma 477
Granular cell tumour 479
Perineurioma 481
Ganglioneuroma and ganglioneuromatosis 483
Tumours of uncertain differentiation
PEComa, including angiomyolipoma 485
Mesenchymal hamartoma of the liver 488
Calcifying nested stromal-epithelial tumour of the liver 490
Synovial sarcoma 492
Gastrointestinal clear cell sarcoma / malignant
gastrointestinal neuroectodermal tumour 494
Embryonal sarcoma of the liver 497
13 Other tumours of the digestive system 499
WHO classification of other tumours of the digestive system 500
Mucosal melanoma 502
Germ cell tumours 504
Metastases 506
14 Genetic tumour syndromes of the digestive system 511
Introduction 512
Lynch syndrome 515
Familial adenomatous polyposis 1 522
GAPPS and other fundic gland polyposes 526
Other adenomatous polyposes 529
Serrated polyposis 532
Hereditary diffuse gastric cancer 535
Familial pancreatic cancer 539
Juvenile polyposis syndrome 542
Peutz–Jeghers syndrome 545
Cowden syndrome 547
Other genetic tumour syndromes 550
Contributors 551
Declaration of interests 557
IARC/WHO Committee for ICD-O 558
Sources 559
References 567
Subject index 625
Previous volumes in the series 635