1 International ICD - O. International Classification of Diseases for Oncology Classification of Diseases for Oncology Third Edition First Revision ISBN 978 92 4 154849 6. ICD-O 1 11/21/13 8:50 AM. ICD - O International Classification of Diseases for Oncology Third Edition First Revision Editors April Fritz Constance Percy Andrew Jack Kanagaratnam Shanmugaratnam Leslie Sobin D Max Parkin Sharon Whelan WHO Library Cataloguing-in-Publication Data International Classification of Diseases for Oncology (ICD-O) 3rd edition, 1st revision. - Classification . Health Organization. ISBN 978 92 4 154849 6 (NLM Classification : QZ 15). ISBN 978 92 4 069212 1 (PDF). World Health Organization 2013. All rights reserved. Publications of the World Health Organization are available on the WHO web site ( ) or can be purchased from WHO Press, World Health Organization, 20 Avenue Appia, 1211 Geneva 27, Switzerland (tel.: +41 22 791 3264.)
2 Fax: +41 22 791 4857; e-mail: Requests for permission to reproduce or translate WHO publications whether for sale or for non-commercial distribution . should be addressed to WHO Press through the WHO web site ( ). The designations employed and the presentation of the material in this publication do not imply the expression of any opinion whatsoever on the part 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. Dotted lines on maps represent approximate border lines for which there may not yet be full agreement. 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.
3 All reasonable precautions have been taken by the World Health Organization to verify the information contained in this publication. However, the published material is being distributed without warranty of any kind, either expressed or implied. The responsibility for the interpretation and use of the material lies with the reader. In no event shall the World Health Organization be liable for damages arising from its use. Printed in Malta Contents Dedication v Acknowledgements vii 1. Introduction 1. Historical background 1. 2. Differences between ICD-O and ICD-10 4. ICD-10 categories not used in ICD-O, third edition 5. Special codes in ICD-O for topography of lymph nodes (C77) and hematopoietic and reticuloendothelial systems (C42) 6. Hydatidiform mole and neurofibromatosis (Von Recklinghausen disease except bone) 6. HIV disease and AIDS 6. Functions of neoplasms 6. 3. Structure and format of ICD-O, third edition 7.
4 Abbreviations 7. American and British spelling 7. Topography numerical list 7. Morphology numerical list 7. Format of ICD-O terms in numerical list 8. Alphabetic index 8. Format and use of alphabetic index 9. Tumor-like lesions and conditions 9. Lymphoma and leukemia listings 9. Meaning of NOS (not otherwise specified) and how it is used 10. Meaning of [OBS] (obsolete) and how it is used 10. The hematologic malignancies 10. Using the lymphoma and leukemia sections of ICD-O 14. 4. Coding guidelines for topography and morphology 14. Summary of principal rules for using ICD-O, third edition 14. Topography 16. Morphology 19. Multiple primary neoplasms 24. Basis of diagnosis 27. WHO grading system for central nervous system tumors and the ICD-O grade code 27. References 29. iii Numerical lists 31. Topography 33. 5th digit behavior code for neoplasms 52. 6th digit code for histological grading and differentiation 52.
5 6th digit code for immunophenotype designation for lymphomas and leukemias 52. Morphology 53. Alphabetic index 95. Appendixes 217. 1: New codes in ICD-O, third edition 217. 2: New morphology terms and synonyms in ICD-O, third edition 222. 3: Terms that changed morphology code in ICD-O, third edition 227. 4: Terms that changed from tumor-like lesions to neoplasms in ICD-O, third edition 230. 5: Terms in ICD-O, second edition, which were deleted for ICD-O, third edition 230. 6: ICD-O, second edition, terms that changed behaviour code for ICD-O, third edition 231. 7: New codes, preferred terms, related terms, and synonyms in this ICD-O, third edition, first revision 233. iv Dedication Calum Muir 1930 1995. This third edition of the International Classification of Diseases for Oncology (ICD-O) is dedicated to the memory of Dr Calum Muir. Calum Muir was an editor of the second edition of ICD-O. As a pathologist, he did much to assemble the new morphologic terms and the latest classifications for lymphomas, leukemias and brain tumors.
6 His contacts with cancer registries throughout the world helped to implement ICD-O worldwide. After his retirement from the International Agency for Research on Cancer, initially as Chief of the Unit of Epidemiology and later as its Deputy Director, Calum Muir became the Director of Cancer Registration for Scotland. He was instrumental in founding the International Association of Cancer Registries (IARC) in 1966, serving as Deputy Secretary from 1972 to 1990 and as President from 1992 until his death. Calum Muir's spirit and devotion to accurate and complete Classification of neoplasms lives on in this edition. iv Acknowledgements We are grateful to the following individuals and their institutions for their contributions to this edition of ICD-O. Dr Timothy Cot , National Cancer Institute, Bethesda, MD, USA. Mme Catherine Exbrayat, Registre des Cancers de l'Is re, Is re, France Professor Ekkehard Grundmann, Gerhard Domagk Institut f r Pathologie, M nster Universit t, M nster, Germany Professor Paul Hermanek, Chirurgische Klinik mit Poliklinik der Universit t Erlangen- N rnberg, Erlangen, Germany Dr Elaine Jaffe, National Cancer Institute, Bethesda, MD, USA.
7 Dr Paul Kleihues, International Agency for Research on Cancer, Lyon, France Dr Franco Rilke, Societ Italiana di Cancerologia, Milan, Italy Dr James Vardiman, University of Chicago, Chicago, IL, USA. Mrs Annette Hurlbut, Fulton, NY, USA. We greatly appreciate the input of the expert groups that provided valuable background material for the development of this third edition and gave the editors the benefit of their advice on the field-trial edition: European Network of Cancer Registries (ENCR) Working Group on the coding of haematological malignancies and lymphomas Dr Ren e Otter, Comprehensive Cancer Centre North, Groningen, The Netherlands Dr Aurora Astudillo, Hospital General de Asturias, Oviedo, Spain Professor Paule Marie Carli, Registre des H mopathies Malignes en C te d'Or, Dijon, France Dr Andrew Jack, Leukaemia Research Fund, University of Leeds, Leeds, England Dr Han Van Krieken, The Academic Hospital, Leiden, The Netherlands International Society of Pediatric Oncology (SIOP) Working Group Professor Jillian M.
8 Birch, CRC Paediatric and Familial Cancer Research Group, Manchester, England Dr James Ironside, National Creutzfeldt-Jakob disease Surveillance Unit, Western General Hospital, Edinburgh, Scotland Dr Anna M. Kelsey, Royal Manchester Children's Hospital, Manchester, England Professor Dietmar Schmidt, Institut f r Pathologie, Mannheim, Germany We would like to thank the cancer registries and the pathologists and data collectors associated with these registries who put an immense amount of careful work into the field testing of ICD-O, third edition. Australia, New South Wales Australia, South Canada, Manitoba Australia, Victoria Canada, Ontario Belarus Canada, Ontario, Princess Margaret Belgium Hospital Brazil, Campinas Canada, Nova Scotia Brazil, Porto Alegre China, Beijing Canada, British Columbia China, Qidong vii International Classification of Diseases , third edition, first revision Cuba Slovenia Czech Republic South Africa Ecuador, Quito Spain, Asturias Egypt, Alexandria Spain, Granada France, Haut-Rhin Spain, Mallorca France, H mopathies Malignes en Spain, Murcia C te d'Or Sweden, Gothenburg France, H rault Thailand, Bangkok France, Tarn Thailand, Chiang Mai Germany, Baden-W rttemberg Trinidad and Tobago Germany, Federal States of Berlin Uganda, Kampala Germany, National Childhood United Kingdom, East Anglia Guinea, Conakry United Kingdom, Northern and Yorkshire Indonesia United Kingdom, Oxford, National Italy, Ferrara Childhood Italy.
9 Macerata USA, Florida, Flagler Hospital Italy, Ragusa USA, Georgia, Medical Center of Central Italy, Romagna Georgia Japan, Hiroshima USA, Missouri, Liberty Hospital Japan, Nagasaki USA, Ohio, Upper Valley Medical Center Japan, Osaka USA (SEER), Atlanta Lithuania USA (SEER), Connecticut Malta USA (SEER), Detroit Netherlands, Amsterdam USA (SEER), Hawaii New Zealand USA (SEER), Iowa Pakistan, Karachi USA (SEER), Los Angeles Peru, Lima USA (SEER), New Mexico Peru, Trujillo USA (SEER), Northern California Philippines, Manila USA (SEER), Seattle/Puget Sound Philippines, Rizal USA (SEER), Utah Poland, Kielce USA, Texas, College Station Medical Saudi Arabia Center Singapore Viet Nam, Ho Chi Minh We also gratefully acknowledge the many individuals, institutions, and registries that took the time to complete the International Association of Cancer Registries' questionnaire and the many registries that offered to participate in the Field Trial when this edition was in development.
10 Viii 1. Introduction principal sites of cancer and includes the morphol- ogy codes of ICD-O for each neoplasm. The International Classification of Diseases for New morphologic terms added since the Oncology (ICD-O) (1) has been used for nearly publication of the second edition are listed at the 35 years, principally in tumor or cancer regis- back of this book. Refractory anemia and other tries, for coding the site (topography) and the myelodysplastic syndromes are now considered to histology (morphology) of the neoplasm, usually be malignant; their behavior codes have therefore obtained from a pathology report. By agreement been changed from /1 (uncertain whether benign with the College of American Pathologists, the or malignant) to /3. Ovarian cystadenomas of bor- morphology section of ICD-O is incorporated derline malignancy which were coded as malignant into the Systematized Nomenclature of Medicine in ICD-O second edition revert to /1 in the third (SNOMED) (2, 3) Classification as the neoplasm edition.