Blaustein's Pathology of the Female Genital Tract Third Edition

Wonderful is he who can teach. . . and wise is he who can be taught.

KATHA- UPANISHAD

Robert J. Kurman Editor

Blaustein's Pathology of the Female Genital Tract Third Edition

With 1176 Illustrations in 1355 Parts, 22 in Full Color

Springer Science+Business Media, LLC

ROBERT J. KURMAN, M.D. Departments of Pathology and Obstetrics and Gynecology Georgetown University School of Medicine Washington, DC 20007, USA

Library of Congress Cataloging in Publication Data Pathology of the female genital tract.

Blaustein's pathology of the female genital tract.

Rev. ed. of: Pathology of the female genital tract / edited by Ancel Blaustein. 2nd ed. cl982.

Includes bibliographies and index. 1. Pathology, Gynecological. I. Blaustein,

Ancel, 1919-1984. II. Kurman, Robert J. III. Title. [DNLM: 1. Genitalia, Female-pathology. WP 100 P297] RG77.P37 1987 618.1'07 86-20357

© 1977, 1982, 1987 by Springer Science+Business Media New York Originally published by Springer-Verlag New York, Inc. in 1987 Softcover reprint of the hardcover 3rd edition 1987

All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher Springer Science+Business Media, LLC. except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use of general descriptive names, trade names, trademarks, etc. in this publication, even if the former are not especially identified, is not to be taken as a sign that such names, as understood by the Trade Marks and Merchandise Marks Act, may accordingly be used freely by anyone. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein.

Typeset by Arcata Graphics/Kingsport Press, Kingsport, Tennessee.

9 8 7 6 5 4 3 2 1

ISBN 978-1-4757-1944-4 ISBN 978-1-4757-1942-0 (eBook) DOl 10.1007/978-1-4757-1942-0

To my parents and to my wife Carole

Preface to the Third Edition

The third edition of this textbook is a continuation of the work begun by Ancel Blaustein a decade ago. The text has been extensively revised to reflect the changes in the field of gynecologic and obstetric pathology since the previ­ous edition. Pathology has not changed but our understanding of it has. New observations lead to the formulation of new criteria and terms that inevitably necessitate a revision of existing classifications. In this regard, the reader is reminded of a statement by Peter Gould l concerning the transient nature of what is considered the "truth" in science, "Whenever we observe and record information about the world around us, we must acknowledge the arbitrary nature of our choices and be aware of the way our choices change over time."

In the last five years application of immunocytochemical and molecular bio­logical techniques to the study of premalignant and malignant lesions of the cervix and vulva has dramatically altered our concepts of neoplasia of the lower female genital tract. Identification of human papillomaviruses in these lesions has led to renewed interest in viral oncogenesis and this has had a major impact on the diagnosis and management of neoplasia in these organs. The chapters on the vulva, vagina, and cervix have been revised accordingly. The chapter on diseases of the vagina has also been expanded to include the changes induced by in utero diethylstilbestrol (DES) exposure, a subject that in the previous edition was considered separately.

The ovary and the endometrium undergo complex alterations as part of the aging process and during the menstrual cycle. The structural changes have been studied by pathologists using light, scanning, and transmission elec­tron microscopy whereas the endocrinologic aspects have been studied by reproductive endocrinologists using biochemical techniques. The chapters on the anatomy and histology of the uterine corpus and ovary describe the morpho­logic changes in the light of serum hormone levels and tissue receptor concen­trations, thereby providing a correlation of the physiology and morphology of these organs.

viii Preface to the Third Edition

In recent years the endometrial proliferations that are precursors of endo­metrial carcinoma as well as lesions that mimic them, i. e., various forms of metaplasia, have been intensively investigated. The histologic features that distinguish the noninvasive precursor lesions from the biologically significant low­grade endometrial carcinomas are now well characterized. In addition, specific histologic types of endometrial carcinoma associated with favorable and unfa­vorable outcomes have been described and their behavior studied. The chapter in the previous edition entitled "Endometrial Neoplasia" has therefore been divided into two chapters, "Endometrial Hyperplasia and Metaplasia" and "Endometrial Carcinoma."

A new chapter entitled "Endometriosis, Lesions of the Secondary Mullerian System, and Pelvic Mesothelial Proliferations" is a unique contribution that presents an in-depth review of endometriosis and develops the concept of the peritoneum as a modified "mullerian-derived organ." This is useful to explain the presence of endometriosis and mullerian-related metaplasias in remote sites throughout the abdomen as well as multifocal extraovarian neopla­sia in the absence of an ovarian tumor.

Clinicopathological studies of the common epithelial, sex cord-stromal, and germ cell tumors of the ovary in the last ten years have greatly influenced their diagnosis and management. The chapter entitled "Sex Cord-Stromal, Steroid Cell, and Other Ovarian Tumors with Endocrine, Paraendocrine, and Paraneoplastic Manifestations" not only summarizes our current under­standing of functioning tumors but also describes a variety of ovarian neo­plasms associated with paraendocrine and paraneoplastic disorders. Some of these entities, such as the small cell carcinoma, have only recently been described.

Gestational trophoblastic disease and germ cell tumors of the ovary represent areas where clinical management has been revolutionized by the use of tumor markers and multiagent chemotherapy. The highly malignant tumors in both of these categories were almost uniformly fatal as recently as fifteen years ago, but are now nearly always cured. It is therefore important for the patholo­gist to recognize these neoplasms and for the clinician to understand their management.

In addition to the content, the format of this edition has been modified to emphasize an integrated presentation of the clinical and pathologic aspects of disease in individual organ systems. Thus, except for the chapters on embryol­ogy, disorders of abnormal sexual development, endometriosis, cytology, and animal tumor models, all the other chapters embrace the spectrum of pathology for a particular organ. For example, infertility is discussed in the chapters on the uterus, fallopian tube, and ovary. Although seemingly fragmented, this approach more closely reflects the pathology of infertility which is commonly categorized into uterine, tubal, and ovarian factors. In instances where a partic­ular disease crosses anatomic boundaries, liberal use of cross-referencing will lead the reader to other parts of the textbook where the subject is discussed. Adjunctive techniques such as electron microscopy and immunocytochemistry are considered in the context of conventional light microscopy and are included where they are useful in diagnosis or in elucidating pathogenesis.

The text is intended to provide an authoritative reference for the practicing obstetrician/gynecologist and pathologist. It is also designed to present a read­able account of gynecologic pathology for medical students and residents in training. The emphasis is on pathophysiology since this provides the foundation for a rational approach to clinical diagnosis and treatment. Students will find that despite the immense accumulation of information in the field, research raises more questions than it answers. Nonetheless, as Esmond R. Long2 stated

Preface to the Third Edition ix

in A History of Pathology, "An honest presentation of pathology compels the constant admission of uncertainty . . . which may lose the student's confi­dence, unless he is made to understand that the whole advancement of the science has been a conquest of similar uncertainties."

ROBERT J. KURMAN, M.D.

References

1. Gould P (1982) The Tyranny of Taxonomy. The Sciences May/June p 7-9 2. Long ER (1965) A History of Pathology. Dover Publications, Inc., New York p

XIII

Preface to the First Edition

This text is written for the obstetrician, gynecologist, pathologist, and for residents training in these disciplines. It is a multiauthored book and the editor is aware of the problems this can create, but the expansion of in­formation in the field of gynecologic pathology renders single authorship obsolete.

The format is largely traditional but the contents include topics that have not appeared in past texts. Clear cell adenocarcinoma of the vagina and vaginal and cervical adenoses are discussed in detail in a separate chapter. A chapter on embryology and congenital anomalies is written by an embryologist and the advantage of its inclusion is self evident. Ovarian neoplasms in childhood and adolescence are fortunately rare occurrences, but information concerning them is generally not readily available in existing texts. It is of sufficient impor­tance to deserve a separate chapter. Amniotic fluid analysis for fetal viability is now commonly used and for this reason a detailed discussion of this subject is presented.

A chapter is included on gross description and preparation of gynecologic specimens. It contains the input and review of several directors of gynecologic­pathology laboratories.

The text contains many electron micrographs taken by transmission and scanning electron microscopy. Their inclusion is not an absolute necessity in gynecologic pathology, but is informative because they offer another perspec­tive and are now a commonly used modality for studying tissue. The present day literature is replete with descriptions of specimens by electron microscopy, and it is hoped that the text will enable the readers to familiarize themselves with electron microscopy as used in this specialty.

Experimentation in the field of obstetrics and gynecology has become more sophisticated over the years and for this reason the chapter on animal models of tumors of the ovaries and uterus in included. The contributions that compara­tive pathology can make to understanding disease mechanisms justify the addi­tion of the chapter on comparative uterine and ovarian tumors in the animal kingdom.

xii Preface to the First Edition

The authors include a mixture of clinicians, pathologists, and basic scientists, and it is hoped that this gives the book the balance between the experience of the clinician and the pathologist.

ANCEL BLAUSTEIN, M.D.

Acknowledgments

I was first introduced to gynecologic pathology by Dr. William B. Ober who I was fortunate to meet and work with during medical school. His influence led me to pursue a career in academic pathology and gynecology and I am grateful to him. My interest in gynecologic pathology was further stimulated during my residency training by Dr. John M. Craig and then by Dr. Robert E. Scully with whom I worked as fellow. To Dr. Scully lowe a debt of gratitude for his continued encouragement, support, and counsel in the suc­ceeding years that I have known him. My association with Dr. Henry J. Norris as a collaborator and close friend has been a source of inspiration throughout my career.

I also wish to acknowledge my distinguished colleagues who contributed to this edition. They not only wrote the scholarly chapters which are the essence of this book but also provided helpful suggestions concerning its overall format and organization. Working closely with them has been an enjoyable and educational experience for me.

I am indebted to Ms. Gail Storey for both her secretarial assistance as well as her managerial skills in organizing the numerous tasks necessary in editing. I am also grateful to the photographic department of the Armed Forces Institute of Pathology, particularly Mr. Luther Duckett, for the excellent photo­micrography that is evident in several of the chapters. The assistance of the staff at Springer-Verlag is also greatly appreciated.

Finally, I wish to pay tribute to the late Dr. Ancel Blaustein, whose vision and creativity led to the creation of Pathology of the Female Genital Tract. The standard of excellence that he set, as well as his wise counsel and infinite patience, were the intangible ingredients that contributed to the success of this book and represent the attributes to which we aspire.

ROBERT J. KURMAN, M.D.

Contents

Preface to the Third Edition

Preface to the First Edition

Acknowledgments

Contributors

1 Embryology of the Female Genital Tract TIM PARMLEY

2 Disorders of Abnormal Sexual Development STANLEY J. ROBBOY, JOSEPH M. LOMBARDO, and WILLIAM R. WELCH

3 Diseases of the Vulva EDWARD J. WILKINSON and EDUARD G. FRIEDRICH, Jr.

4 Diseases of the Vagina ALEX SEDLIS and STANLEY J. ROBBOY

5 Anatomy and Histology of the Cervix ALEX FERENCZY and BARBARA WINKLER

6 Benign Diseases of the Cervix ALEX FERENCZY and BARBARA WINKLER

7 Cervical Intraepithelial Neoplasia and Condyloma ALEX FERENCZY and BARBARA WINKLER

8 Carcinoma and Metastatic Tumors of the Cervix ALEX FERENCZY and BARBARA WINKLER

9 Anatomy and Histology of the Uterine Corpus ALEX FERENCZY

10 Benign Diseases of the Endometrium ROBERT J. KURMAN and MICHAEL T. MAZUR

vii

xi

xiii

xvii

1

15

36

97

141

158

177

218

257

292

xvi Contents

11 Endometrial Hyperplasia and Metaplasia ROBERT J. KURMAN and HENRY J. NORRIS

12 Endometrial Carcinoma ROBERT J. KURMAN and HENRY J. NORRIS

13 Mesenchymal Tumors of the Uterus CHARLES ZALOUDEK and HENRY J. NORRIS

14 Diseases of the Fallopian Tube JAMES E. WHEELER

15 Anatomy and Histology of the Ovary PHILIP B. CLEMENT

16 Nonneoplastic Lesions of the Ovary PHILIP B. CLEMENT

17 Endometriosis, Lesions of the Secondary Mullerian System, and Pelvic Mesothelial Proliferations PHILIP B. CLEMENT

18 Common Epithelial Tumors of the Ovary BERNARD CZERNOBILSKY

19 Sex Cord-Stromal, Steroid Cell, and Other Ovarian Tumors with Endocrine, Paraendocrine, and Paraneoplastic Manifestations ROBERT H. YOUNG and ROBERT E. SCULLY

20 Germ Cell Tumors of the Ovary ALEKSANDER TALERMAN

21 Nonspecific Tumors of the Ovary, Including Mesenchymal Tumors and Malignant Lymphoma ALEKSANDER T ALERMAN

22 Metastatic Tumors of the Ovary ROBERT H. YOUNG and ROBERT E. SCULLY

23 Diseases of the Placenta DEBORAH J. GERSELL, FREDERICK T. KRAUS, and MAUREEN BURKE RIFFLE

24 Gestational Trophoblastic Disease MICHAEL T. MAZUR and ROBERT J. KURMAN

25 Cytology of the Female Genital Tract RITA LEFF BLAUSTEIN

26 Animal Models for Tumors of the Female Genital Tract JUNE MARCHANT

27 Gross Description, Processing, and Reporting of

322

338

373

409

438

471

516

560

607

659

722

742

769

835

876

899

Gynecologic and Obstetric Specimens 925 STANLEY T. ROBBOY, FREDERICK T. KRAUS, and ROBERT J. KURMAN

Index 941

Contributors

RITA LEFF BLAUSTEIN, M.D. Attending Cytopathologist Department of Pathology Booth Memorial Medical Center Flushing, New York, USA

PHILIP B. CLEMENT, M.D., F.R.C.P.(C) Clinical Professor of Pathology University of British Columbia Consulting Pathologist Vancouver General Hospital Vancouver, British Columbia, Canada

BERNARD CZERNOBILSKY, M.D. Professor of Pathology Medical School of the Hebrew University and

Hadassah Jerusalem, Israel Chief Department of Pathology Kaplan Hospital Rehovot, Israel

ALEX FERENCZY, M.D. Professor of Pathology and Obstetrics and Gynecology McGill University and The Sir Mortimer B. Davis

Jewish General Hospital Head Gynecologic Pathology and Cytology and Colposcopy The Sir Mortimer B. Davis Jewish General Hospital Montreal, Quebec, Canada

EDUARD H. FRIEDRICH, JR., M.D., L.L.D. W.C. Thomas Professor Department of Obstetrics and Gynecology University of Florida School of Medicine Gainesville, Florida, USA

DEBORAH J. GERSELL, M. D. Assistant Professor of Pathology Washington University School of Medicine Assistant Pathologist Barnes Hospital St. Louis, Missouri, USA

FREDERICK T. KRAus, M. D. Professor, Pathology (Visiting Staff) Washington University School of Medicine Director Laboratory Medicine St. John's Mercy Medical Center St. Louis, Missouri, USA

ROBERT J. KURMAN, M.D. Professor of Pathology and Obstetrics and

Gynecology Georgetown University School of Medicine Director Gynecologic Pathology Georgetown University Hospital Washington, D.C., USA

xviii Contributors

JOSEPH M. LOMBARDO, M.D., PH.D. Assistant Professor of Pathology New Jersey Medical School University of Medicine and Dentistry of New Jersey Director of Clinical Immunovirology University Hospital Newark, New Jersey, USA

JUNE MARCHANT, PH.D. Emeritus Senior Lecturer in the Biology of Cancer University of Birmingham Birmingham, England

MICHAEL T. MAZUR, M.D. Associate Professor of Pathology University of Alabama at Birmingham Surgical Pathologist University of Alabama Hospital Birmingham, Alabama, USA

HENRY J. NORRIS, M.D. Clinical Professor of Pathology Uniformed Services University of the Health Sciences Bethesda, Maryland Chairman Department of Gynecologic and Breast Pathology Armed Forces Institute of Pathology Washington, D.C., USA

TIM PARMLEY, M.D. Associate Professor of Gynecology and Obstetrics Johns Hopkins University School of Medicine Obstetrician and Gynecologist Johns Hopkins Hospital Baltimore, Maryland, USA

MAUREEN BURKE RIFFLE, M.D. Pathologist De Paul Community Health Center St. Louis, Missouri, USA

STANLEY J. ROBBOY, M.D. Chairman and Professor of Pathology New Jersey Medical School University of Medicine and Dentistry of New Jersey Chief Department of Pathology University Hospital Newark, New Jersey, USA

ROBERT E. SCULLY, M.D. Professor of Pathology Harvard Medical School Pathologist Massachusetts General Hospital Boston, Massachusetts, USA

ALEXANDER SEDLIS, M. D. Professor of Obstetrics and Gynecology State University of New York, Health Science Center

at Brooklyn Director Department of Gynecology Kings County Medical Center Health Science Center at Brooklyn Brooklyn, New York, USA

ALEKSANDER TALERMAN, M.D., PH.D., F.R.C. PATH. Professor of Pathology and Obstetrics and

Gynecology University of Chicago Chicago, Illinois, USA

WILLIAM R. WELCH, M.D. Associate Professor of Pathology Harvard Medical School Chief Gynecologic Surgical Pathology Brigham and Women's Hospital Boston, Massachusetts, USA

JAMES E. WHEELER, M. D. Professor Departments of Pathology and Laboratory Medicine

and Obstetrics and Gynecology Hospital of the University of Pennsylvania Philadelphia, Pennsylvania, USA

EDWARD J. WILKINSON, M.D. Professor of Pathology and Obstetrics and Gynecology University of Florida School of Medicine Director of the Section of Gynecologic Pathology and

Cytopathology Shands Hospital Gainesville, Florida, USA

BARBARA WINKLER, M.D. Assistant Professor of Pathology and Obstetrics,

Gynecology and Reproductive Sciences University of California Director Obstetrical and Gynecological Pathology Service Co-Director, Immunopathology Service University of California at San Francisco Medical

Center San Francisco, California, USA

ROBERT H. YOUNG, M.D., M.R.C.PATH.

Associate Professor of Pathology Harvard Medical School Associate Pathologist Massachusetts General Hospital Boston, Massachusetts, USA

CHARLES J. ZALOUDEK, M.D.

Anatomic Pathologist Alta Bates Hospital Berkeley, California, USA

Contributors xix