Pathology and Genetics of Tumours of the Digestive System
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BB2 Title pages 1-8 Digestive.qxd 18.7.2006 16:08 Page 3 World Health Organization Classification of Tumours WHO OMS International Agency for Research on Cancer (IARC) Pathology and Genetics of Tumours of the Digestive System Edited by Stanley R. Hamilton Lauri A. Aaltonen IARCPress Lyon, 2000 BB2 Title pages 1-8 Digestive.qxd 18.7.2006 16:08 Page 4 World Health Organization Classification of Tumours Series Editors Paul Kleihues, M.D. Leslie H. Sobin, M.D. Pathology and Genetics of Tumours of the Digestive System Editors Stanley R. Hamilton, M.D. Lauri A. Aaltonen, M.D., Ph.D. Clinical Editor René Lambert, M.D Editorial Assistance Wojciech Biernat, M.D. Norman J. Carr, M.D. Anna Sankila, M.D. Layout Sibylle Söring Felix Krönert Illustrations Georges Mollon Sibylle Söring Printed by Team Rush 69603 Villeurbanne, France Publisher IARCPress International Agency for Research on Cancer (IARC) 69372 Lyon, France BB2 Title pages 1-8 Digestive.qxd 18.7.2006 16:08 Page 5 This volume was produced in collaboration with the International Academy of Pathology (IAP) and with support from the Swiss Federal Office of Public Health, Bern The WHO Classification of Tumours of the Digestive System presented in this book reflects the views of a Working Group that convened for an Editorial and Consensus Conference in Lyon, France, November 6-9, 1999. Members of the Working Group are indicated in the List of Contributors on page 253. BB2 Title pages 1-8 Digestive.qxd 18.7.2006 16:08 Page 6 Published by IARC Press, International Agency for Research on Cancer, 150 cours Albert Thomas, F-69372 Lyon, France © International Agency for Research on Cancer, 2000 reprinted 2006 Publications of the World Health Organization enjoy copyright protection in accordance with the provisions of Protocol 2 of the Universal Copyright Convention. All rights reserved. The International Agency for Research on Cancer welcomes requests for permission to reproduce or translate its publications, in part or in full. Requests for permission to reproduce figures or charts from this publication should be directed to the respective contributor (see section Source of Charts and Photographs). The designations used and the presentation of the material in this publication do not imply the expression of any opinion whatsoever on the part of the Secretariat of the World Health Organization concerning the legal status of any country, territory, city, or area or of its authorities, or concerning the delimitation of its frontiers or boundaries. The mention of specific companies or of certain manufacturers' products does not imply that they are endorsed or recommended by the World Health Organization in preference to others of a similar nature that are not mentioned. Errors and omissions excepted, the names of proprietary products are distinguished by initial capital letters. The authors alone are responsible for the views expressed in this publication. Enquiries should be addressed to the Editorial & Publications Service, International Agency for Research on Cancer, 69372 Lyon, France, which will provide the latest information on any changes made to the text and plans for new editions. Format for bibliographic citations: Hamilton S.R., Aaltonen L.A. (Eds.): World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System. IARC Press: Lyon 2000 IARC Library Cataloguing in Publication Data Pathology and genetics of tumours of the digestive system / editors, S.R. Hamilton and L.A. Aaltonen (World Health Classification of tumours ; 2) 1. Digestive System Neoplasms I. Aaltonen, L.A. II. Hamilton, S.R. III. Series ISBN 92 832 2410 8 (NLM Classification: W1) BB2 Title pages 1-8 Digestive.qxd 18.7.2006 16:08 Page 7 Contents Diagnostic terms and definitions 8 Endocrine tumours 137 B-cell lymphoma 139 Mesenchymal tumours 142 1 Tumours of the oesophagus 9 WHO and TNM classifications 10 Squamous cell carcinoma 11 7 Tumours of the anal canal 145 Adenocarcinoma 20 WHO and TNM classifications 146 Endocrine tumours 26 Tumours of the anal canal 147 Lymphoma 27 Mesenchymal tumours 28 Secondary tumours and melanoma 30 8 Tumours of the liver and intrahepatic bile ducts 157 WHO and TNM classifications 158 2 Tumours of the oesophagogastric junction 31 Hepatocellular carcinoma 159 Adenocarcinoma 32 Intrahepatic cholangiocarcinoma 173 Combined hepatocellular and cholangiocarcinoma 181 Bile duct cystadenoma and cystadenocarcinoma 182 3 Tumours of the stomach 37 Hepatoblastoma 184 WHO and TNM classifications 38 Lymphoma 190 Carcinoma 39 Mesenchymal tumours 191 Endocrine tumours 53 Secondary tumours 199 Lymphoma 57 Mesenchymal tumours 62 Secondary tumours 66 9 Tumours of the gallbladder and extrahepatic bile ducts 203 WHO and TNM classifications 204 4 Tumours of the small intestine 69 Carcinoma 206 WHO and TNM classifications 70 Endocrine tumours 214 Carcinoma 71 Neural and mesenchymal tumours 216 Peutz-Jeghers syndrome 74 Lymphoma 217 Endocrine tumours 77 Secondary tumours and melanoma 217 B-cell lymphoma 83 T-cell lymphoma 87 Mesenchymal tumours 90 10 Tumours of the exocrine pancreas 219 Secondary tumours 91 WHO and TNM classifications 220 Ductal adenocarcinoma 221 Serous cystic neoplasms 231 5 Tumours of the appendix 93 Mucinous cystic neoplasms 234 WHO and TNM classifications 94 Intraductal papillary-mucinous neoplasm 237 Adenocarcinoma 95 Acinar cell carcinoma 241 Endocrine tumours 99 Pancreatoblastoma 244 Miscellaneous tumours 102 Solid-pseudopapillary neoplasm 246 Miscellaneous carcinomas 249 Mesenchymal tumours 249 6 Tumours of the colon and rectum 103 Lymphoma 250 WHO and TNM classifications 104 Secondary tumours 250 Carcinoma 105 Familial adenomatous polyposis 120 Contributors 253 Hereditary nonpolyposis colorectal cancer 126 Juvenile polyposis 130 Source of charts and photographs 261 Cowden syndrome 132 References 265 Hyperplastic polyposis 135 Subject index 307 BB2 Title pages 1-8 Digestive.qxd 18.7.2006 16:08 Page 8 Diagnostic terms and definitions1 Intraepithelial neoplasia2. A lesion cha- Tubulovillous adenoma. An adenoma Mucinous adenocarcinoma. An ade- racterized by morphological changes composed of both tubular and villous nocarcinoma containing extracellular that include altered architecture and structures, each comprising more than mucin comprising more than 50% of the tumour. Note that ‘mucin producing’ is abnormalities in cytology and differentia- 20% of the tumour. tion. It results from clonal alterations in not synonymous with mucinous in this context. genes and carries a predisposition for Serrated adenoma. An adenoma com- progression to invasion and metastasis. posed of saw-toothed glands. Signet-ring cell carcinoma. An adeno- carcinoma in which the predominant High-grade intraepithelial neoplasia. Intraepithelial neoplasia (dysplasia) component (more than 50%) is com- A mucosal change with cytologic and associated with chronic inflammatory posed of isolated malignant cells con- architectural features of malignancy but taining intracytoplasmic mucin. diseases. A neoplastic glandular without evidence of invasion into the stro- epithelial proliferation occurring in a ma. It includes lesions termed severe Squamous cell (epidermoid) carcino- patient with a chronic inflammatory dysplasia and carcinoma in situ. ma. A malignant epithelial tumour with bowel disease, but with macroscopic squamous cell differentiation. and microscopic features that distin- Polyp. A generic term for any excres- Adenosquamous carcinoma. A malig- guish it from an adenoma, e.g. patchy cence or growth protruding above a nant epithelial tumour with significant mucous membrane. Polyps can be distribution of dysplasia and poor cir- components of both glandular and squa- pedunculated or sessile, and are readily cumscription. mous differentiation. seen by macroscopic examination or conventional endoscopy. Peutz-Jeghers polyp. A hamartoma- Small cell carcinoma. A malignant epithelial tumour similar in morphology, tous polyp composed of branching immunophenotype and behaviour to Adenoma. A circumscribed benign bands of smooth muscle covered by nor- small cell carcinoma of the lung. lesion composed of tubular and/or villous mal-appearing or hyperplastic glandular structures showing intraepithelial neopla- mucosa indigenous to the site. Medullary carcinoma. A malignant sia. The neoplastic epithelial cells are epithelial tumour in which the cells form immature and typically have enlarged, Juvenile polyp. A hamartomatous solid sheets and have abundant hyperbasophilic and stratified nuclei. polyp with a spherical head composed eosinophilic cytoplasm and large, vesic- ular nuclei with prominent nucleoli. An of tubules and cysts, lined by normal Tubular adenoma. An adenoma in intraepithelial infiltrate of lymphocytes is epithelium, embedded in an excess of which branching tubules surrounded by characteristic. lamina propria comprise at least 80% of lamina propria. In juvenile polyposis, the Undifferentiated carcinoma. A malig- the tumour. polyps are often multilobated with a pap- illary configuration and a higher ratio of nant epithelial tumour with no glandular structures or other features to indicate Villous adenoma. An adenoma in which glands to lamina propria. definite differentiation. leaf-like or finger-like processes of lami- na propria covered by dysplastic epithe- Adenocarcinoma. A malignant epithe- Carcinoid. A well differentiated neo- lium comprise at least 80% of the tumour. lial tumour with glandular differentiation. plasm of the diffuse endocrine system. ______________