Mesenchymal tumours - International Agency for Research on Cancer

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World Health Organization Classification of Tumours
WHO OMS
International Agency for Research on Cancer (IARC)
Pathology and Genetics of
Tumours of the Breast and
Female Genital Organs
Edited by
Fattaneh A. Tavassoli
Peter Devilee
IARCPress
Lyon, 2003
World Health Organization Classification of Tumours
Series Editors Paul Kleihues, M.D.
Leslie H. Sobin, M.D.
Pathology and Genetics of Tumours of the Breast and Female Genital Organs
Editors Fattaneh A. Tavassoli, M.D.
Peter Devilee, Ph.D.
Coordinating Editors Lawrence M. Roth, M.D.
Rosemary Millis, M.D.
Editorial Assistants Isabelle Forcier
Christine Zorian
Layout Lauren A. Hunter
Sibylle Söring
Pascale Dia
Illustrations Georges Mollon
Lauren A. Hunter
Printed by Druckhaus Tecklenborg
48565 Steinfurt, Germany
Publisher IARCPress
International Agency for
Research on Cancer (IARC)
69008 Lyon, France
This volume was produced in collaboration with the
International Academy of Pathology (IAP)
The WHO Classification of Tumours of the Breast and Female Genital Organs
presented in this book reflects the views of Working Groups that convened for
Editorial and Consensus Conferences in Lyon, France,
January 12-16 and March 16-20, 2002.
Members of the Working Groups are indicated
in the List of Contributors on page 365
The Working Group on Gynaecological Tumours greatly
appreciates the participation of, and guidance by
Dr. Robert E. Scully, Harvard Medical School
Published by IARC Press, International Agency for Research on Cancer,
150 cours Albert Thomas, F-69008 Lyon, France
© International Agency for Research on Cancer, 2003
Publications of the World Health Organization enjoy copyright protection in
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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).
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Enquiries should be addressed to the
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Format for bibliographic citations:
Tavassoli F.A., Devilee P.(Eds.): World Health Organization Classification of
Tumours. Pathology and Genetics of Tumours of the Breast
and Female Genital Organs. IARC Press: Lyon 2003
IARC Library Cataloguing in Publication Data
Pathology and genetics of tumours of the breast and female genital organs /
editors, Fattaneh A. Tavassoli, Peter Devilee.
(World Health Organization classification of tumours ; 5)
1. Breast Neoplasms—genetics. 2. Breast Neoplasms—pathology
3. Genital Neoplasms, Female—genetics 4. Genital Neoplasms, Female—pathology
I. Tavassoli, Fattaneh A. II. Devilee, Peter III. Series
ISBN 92 832 2412 4 (NLM Classification W 1)
Contents
1 Tumours of the breast 9 3 Tumours of the fallopian tube and
Invasive breast carcinoma 13 uterine ligaments 203
Invasive ductal carcinoma, NOS 19 Tumours of the fallopian tube 206
Invasive lobular carcinoma 23 Tumours of the uterine ligaments 212
Tubular carcinoma 26
Invasive cribriform carcinoma 27 4 Tumours of the uterine corpus 217
Medullary carcinoma 28 Epithelial tumours and related lesions 221
Mucin producing carcinomas 30 Endometrial carcinoma 221
Neuroendocrine tumours 32 Endometrial hyperplasia 228
Invasive papillary carcinoma 34 Endometrial polyp 230
Mesenchymal tumours and related lesions 233
Invasive micropapillary carcinoma 35
Endometrial stromal and related tumours 233
Apocrine carcinoma 36
Smooth muscle tumours 236
Metaplastic carcinomas 37
Other mesenchymal tumours 242
Lipid-rich carcinoma 41
Mixed epithelial and mesenchymal tumours 245
Secretory carcinoma 42
Gestational trophoblastic disease 250
Oncocytic carcinoma 43
Sex cord-like, neuroectodermal / neuroendocrine tumours,
Adenoid cystic carcinoma 44
lymphomas and leukaemias 255
Acinic cell carcinoma 45
Secondary tumours of the uterine corpus 257
Glycogen-rich clear cell carcinoma 46
Sebaceous carcinoma 46 5 Tumours of the uterine cervix 259
Inflammatory carcinoma 47 Epithelial tumours 262
Bilateral breast carcinoma 48 Squamous tumours and precursors 266
Precursor lesions Glandular tumours and precursors 272
Lobular neoplasia 60 Uncommon carcinomas and neuroendocrine tumours 277
Intraductal proliferative lesions 63 Mesenchymal tumours 280
Microinvasive carcinoma 74 Mixed epithelial and mesenchymal tumours 284
Intraductal papillary neoplasms 76 Melanotic, germ cell, lymphoid and
Benign epithelial lesions 81 secondary tumours of the cervix 287
Myoepithelial lesions 86
Mesenchymal tumours 89 6 Tumours of the vagina 291
Fibroepithelial tumours 99 Epithelial tumours 293
Tumours of the nipple 104 Squamous tumours 293
Malignant lymphoma and metastatic tumours 107 Glandular tumours 297
Tumours of the male breast 110 Tumours of skin appendage origin 324
Mesenchymal tumours 302
2 Tumours of the ovary and peritoneum 113 Mixed epithelial and mesenchymal tumours 306
Melanotic, neuroectodermal, lymphoid and
Surface epithelial-stromal tumours 117
secondary tumours 308
Serous tumours 119
Mucinous tumours 124 7 Tumours of the vulva 313
Endometrioid tumours 130 Epithelial tumours 316
Clear cell tumours 137 Squamous tumours 316
Transitional cell tumours 140 Glandular tumours 321
Squamous cell lesions 143 Mesenchymal tumours 326
Mixed epithelial tumours 144 Melanocytic tumours 331
Undifferentiated carcinomas 145 Germ cell, neuroectodermal, lymphoid and
Sex cord-stromal tumours 146 secondary tumours 333
Granulosa-stromal cell tumours 146 8 Inherited tumour syndromes 335
Sertoli-stromal cell tumours 153 Familial aggregation of cancers of the breast and
Mixed sex cord-stromal tumours 158 female genital organs 336
Steroid cell tumours 160 BRCA1 syndrome 338
Germ cell tumours 163 BRCA2 syndrome 346
Primitive germ cell tumours 163 Li-Fraumeni syndrome 351
Biphasic or triphasic teratomas 168 Cowden syndrome 355
Monodermal teratomas 171 Hereditary non-polyposis colon cancer (HNPCC) 358
Mixed germ cell-sex cord-stromal tumours 176 Ataxia telangiectasia syndrome 361
Tumours and related lesions of the rete ovarii 180
Miscellaneous tumours and tumour-like lesions 182 Contributors 365
Lymphomas and leukaemias 191 Source of charts and photographs 370
Secondary tumours of the ovary 193 References 372
Peritoneal tumours 197 Subject index 425
CHAPTER 1
Tumours of the Breast
Cancer of the breast is one of the most common human neo-
plasms, accounting for approximately one quarter of all can-
cers in females. It is associated with the Western lifestyle, and
incidence rates are, there f o re, highest in countries with
advanced economies. Additional risk factors include early
menarche and late childbirth. Breast cancer is further charac-
terized by a marked genetic susceptibility. Early detection and
advances in treatment have begun to reduce mortality rates in
several countries. Through the use of cDNA expression pro-
files, it may become possible to predict clinical outcome in indi-
vidual patients.
The typing of invasive breast cancer and its histological vari-
ants is well established. More difficult is the classification of
pre-invasive breast lesions which are now increasingly detect-
ed by mammography. The WHOWorking Group agreed that
more clinical follow-up and genetic data are needed for a bet-
ter understanding of the natural history of these lesions.