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The book provides a description at length of the epidemiology, etiology, pathological anatomy, prognosis, diagnosis, differential diagnosis, imaging and comprehensive therapy of each primary, relapsed, and metastasized tumor including surgery, chemo-, hormone- and radio- and targeted therapy. Günter Köhler, Matthias Evert, Katja Evert, Marek Zygmunt Sarcoma of the female genitalia Günter Köhler, Matthias Evert, Katja Evert, Marek Zygmunt Sarcoma of the female genitalia | Volume 1: Smooth muscle and stromal tumors and prevention of inadequate sarcoma surgery In collaboration with Philipp-Andreas Hessler, Lars Kaderali, Hanka Lehnhoff, Lisa Linke Authors Prof Dr Günter Köhler Department of Obstetrics and Gynecology, University Medicine Greifswald and German Clinical Center of Excellence for Genital Sarcomas and Mixed Tumors Ferdinand-Sauerbruch-Straße 17475 Greifswald, Germany e-mail: koehlerg@uni-greifswald.de Prof Dr Matthias Evert Institute of Pathology, University Hospital Regensburg and German Clinical Center of Excellence for Genital Sarcomas and Mixed Tumors, Greifswald Franz-Josef-Strauß-Allee 11 93053 Regensburg, Deutschland e-mail: matthias.evert@ukr.de Prof Dr Marek Zygmunt Department of Obstetrics and Gynecology, University Medicine Greifswald and German Clinical Center of Excellence for Genital Sarcomas and Mixed Tumors Ferdinand-Sauerbruch-Straße 17475 Greifswald, Germany e-mail: zygmunt@uni-greifswald.de Dr Katja Evert Institute of Pathology, University Hospital Regensburg and German Clinical Center of Excellence for Genital Sarcomas and Mixed Tumors, Greifswald Franz-Josef-Strauß-Allee 11 93053 Regensburg, Deutschland e-mail: katja.evert@ukr.de Collaborators Dr Philipp-Andreas Hessler Department of Gynecologic Surgery, Center of Minimal-Invasive Surgery, Sachsenhausen, Frankfurt/Main/Germany Prof Dr Lars Kaderali Institute for Bioinformatics, University Medicine Greifswald/Germany Hanka Lehnhoff Department of Obstetrics and Gynecology University Medicine Greifswald, Germany and German Clinical Center of Excellence for Genital Sarcomas and Mixed Tumors, Greifswald/Germany Lisa Linke Department of Obstetrics and Gynecology University Medicine Greifswald and German Clinical Center of Excellence for Genital Sarcomas and Mixed Tumors, Greifswald/Germany ISBN 978-3-11-035141-5 e-ISBN (PDF) 978-3-11-035197-2 e-ISBN (EPUB) 978-3-11-038763-6 The publisher, together with the authors and editors, has taken great pains to ensure that all information presented in this work (programs, applications, amounts, dosages, etc.) reflects the standard of knowledge at the time of publication Despite careful manuscript preparation and proof correction, errors can nevertheless occur Authors, editors and publisher disclaim all responsibility and for any errors or omissions or liability for the results obtained from use of the information, or parts thereof, contained in this work The citation of registered names, trade names, trademarks, etc in this work does not imply, even in the absence of a specific statement, that such names are exempt from laws and regulations protecting trademarks etc and therefore free for general use Library of Congress Cataloging-in-Publication Data A CIP catalog record for this book has been applied for at the Library of Congress Bibliographic information published by the Deutsche Nationalbibliothek The Deutsche Nationalbibliothek lists this publication in the Deutsche Nationalbibliografie; detailed bibliographic data are available on the Internet at http://dnb.dnb.de © 2017 Walter de Gruyter GmbH, Berlin/Boston Cover image: Hackelöer HJ, Kreiselmaier P Facharzt-Zentrum für Kinderwunsch Pränatale Medizin, Endokrinologie u Osteologie, Amedes Experts Hamburg, Hamburg, Germany Translation into English: Dr jur Philip Horsfield, Greifswald/Germany ⟨translate@philiphorsfield.com⟩ Typesetting: PTP-Berlin, Protago-TEX-Production GmbH, Berlin Printing and binding: Hubert & Co GmbH und Co KG, Göttingen ♾ Printed on acid-free paper Printed in Germany www.degruyter.com Foreword This monograph, comprising two volumes, discusses typical sarcomas of the female genitalia, including angiosarcoma, liposarcoma and rhabdomyosarcoma as well as the different types of mixed tumors Other rare mesenchymal genital tumors are also comprehensively presented, such as the different variants of leiomyoma, smooth muscle tumors with uncertain malignant potential, endometrial stromal nodules, endometrial stromal tumors with sex cord-like elements (ESTSCLE), uterine tumor resembling ovarian sex-cord/stromal tumor (UTROCST) and PEComas The behavior of the aforementioned neoplasms in relation to, and in the context of fertility and pregnancy is covered in a separate chapter Another chapter is devoted to the prevention of subjecting sarcomas to inadequate surgical therapeutic measures under the assumed diagnosis of leiomyoma, and includes a diagnostic-therapeutic flowchart with a diagnostic score The following subsections are provided for each of the tumor entities listed above: epidemiology, etiology, macroscopy and microscopy, clinical presentation, diagnostics, imaging, differential diagnostics, prognosis, surgical and radiation therapy for the primary tumor or recurrences and metastases including primary, adjuvant and palliative chemotherapy, hormone therapy, targeted therapy and aftercare The substantial basis for this monograph comprises 1,681 fully documented sarcoma consultation cases of the German Clinical Center of Excellence for Genital Sarcomas and Mixed Tumors (“Deutsches klinisches Kompetenzzentrum für genitale Sarkome und Mischtumoren”, DKSM), University Medicine Greifswald/Germany, and the data kindly provided by our cooperation partners “Velener Working Group on Ambulatory Surgery” (“Velener Arbeitsgemeinschaft Ambulantes Operieren Deutschland”, VAAO) and the Department Gynecologic Surgery, Center of Minimal-Invasive Surgery, Sachsenhausen/Frankfurt Main/Germany The overriding aim of this monograph is to identify and provide therapeutic guidance on the basis of evaluation of the DKSM data performed by the DKSM doctoral research group “uterine sarcomas”, as well as on 2,025 literature sources, recommendations and studies published up to the end of May 2016 Particular attention has been devoted to determining, as far as possible, which measures can currently not be defined as standard practice In total, 576 figures and illustrations have been incorporated and are comprehensively described The listed tumor entities also constitute a particular diagnostic challenge for pathologists that is beset with numerous pitfalls and difficulties This monograph, therefore, addresses gynecologists and pathologists in both clinical and private practice, but also surgeons and hemato-oncologists VI | Foreword Sincere thanks must be extended to a total of 112 gynecologists, pathologists, radiologists, hemato-oncologists and medical practitioners in clinical and private practice, but also affected patients, who kindly provided figures, images and data for use in this monograph Greifswald and Regensburg, June 2016 Prof Dr G Köhler, Prof Dr M Zygmunt, Department of Obstetrics and Gynecology, University Medicine Greifswald and German Clinical Center of Excellence for Sarcomas and Mixed Tumors, Greifswald Prof Dr M Evert, Dr Katja Evert Institute of Pathology, University Hospital Regensburg and German Clinical Center of Excellence for Genital Sarcomas and Mixed Tumors, Greifswald Sarcoma of the female genitalia Volume 1: Smooth muscle and stromal tumors and prevention of inadequate sarcoma surgery (ISBN: 978-3-11-035141-5) Volume 2: Other rare sarcomas, mixed tumors, genital sarcomas and pregnancy (ISBN: 978-3-11-045921-0) Contents Foreword | V List of abbreviations | XXI Definition of cytoreduction, NCCN-Guidelines | XXV Immunohistochemistry table | XXVI Volume Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert Variants of leiomyoma (angio- and lipoleiomyoma, cotyledonoid and cellular leiomyoma, leiomyoma with bizarre nuclei, mitotically active, epithelioid and myxoid leiomyoma), smooth muscle tumors with uncertain malignant potential (atypical smooth muscle tumors), disseminated peritoneal leiomyomatosis, benign metastasizing leiomyoma, intravenous leiomyomatosis | 1.1 Angioleiomyoma (Angiomyoma, vascular leiomyoma) | 1.1.1 General, epidemiology, etiology, pathogenesis, pathological-anatomical findings | 1.1.2 Clinical presentation, diagnostics, imaging, differential diagnostics | 1.1.3 Course, prognosis, primary surgery, systemic and radiogenic therapy | 1.1.4 Aftercare, recurrences, metastases and their treatment | 1.2 Lipoleiomyoma | 1.2.1 General, pathogenesis, pathological-anatomical findings | 1.2.2 Clinical presentation, diagnostics, imaging, differential diagnostics | 1.2.3 Course, prognosis, primary surgery, systemic and radiogenic therapy | 1.3 Cotyledonoid dissecting leiomyoma | 1.3.1 General, pathogenesis, pathological-anatomical findings | 1.3.2 Clinical presentation, diagnostics, imaging, differential diagnostics | 1.3.3 Course, prognosis, primary surgery, systemic and radiogenic therapy | 216 | Leiomyosarcoma (125) (126) (127) (128) (129) (130) (131) (132) (133) (134) (135) 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2.1.4 (A), (C)–(I); 2.1.7 (A); 2.1.13; 2.1.16 (A); 2.1.18 (B); 2.2.2 (C) Glaubitz M Klinikum Nordfriesland, Klinik Husum, Gynäkologie, Husum Fig 2.1.7 (B) Grimminger HJ, Missionsärztliche Klinik Würzburg, Gynäkologie und Geburtshilfe, Würzburg Fig 2.6.1 (A) Gürtler F Kreiskrankenhaus Wolgast, Abt Gynäkologie u Geburtshilfe, Wolgast Fig 2.1.1 (B) Heidkamp L Albertinen Krankenhaus Hamburg, Albertinen Frauenkliniken, Hamburg Fig 2.1.2 (D), (E) Hessler PA, Kuhfus S, Dennis K Krankenhaus Sachsenhausen, operative Gynäkologie, Frankfurt/Main Fig 2.1.1 (F); 2.1.9 (C), (D); 2.1.15 (A), (B) Hosten N, Hegenscheid K Universitätsmedizin Greifswald, Institut für Diagnostische Radiologie und Neuroradiologie, Greifswald Fig 2.1.1 (C); 2.1.10 (A)–(D); 2.1.19 (A), (D) Holthaus B Kardinal von Galen Kliniken, Krankenhaus ST Elisabeth, Gynäkologie, Damme Fig 2.1.8 (A)–(C) References | 229 Köhler G, Zygmunt M Universitätsmedizin Greifswald, Klinik Frauenheilkunde und Geburtshilfe, Greifswald Fig 2.1.1 (A), (D), (E); 2.1.2 (C); 2.1.4 (B); 2.1.11 (A), (B); 2.1.15 (E), (F); 2.1.16 (B)–(G) Krombach G Universitätsklinikum Gießen und Marburg, Diagnostische Radiologie, Gießen Fig 2.1.10 (E) Krystek E St Josefskrankenhaus Heidelberg, Gynäkologie und Geburtshilfe, Heidelberg Fig 2.1.2 (B) Kurz C Klinikum Esslingen, Klinik für Frauenheilkunde und Geburtshilfe, Esslingen Fig 2.1.5 (A), (B) Lampe B, Gröning T Florence-Nightingale-Krankenhaus, Klinik für Gynäkologie u Geburtshilfe, Düsseldorf-Kaiserswerth Fig 2.1.7 (G) Lehnhardt M Berufsgenossenschaftliches Univ.-Klinikum Bergmannsheil, Klinik f plastische Chirurgie, Bochum Fig 2.1.18 (C) Martin B Kliniken Landkreis Tuttlingen, Frauenklinik, Tuttlingen Fig 2.1.15 (C) Maurer C St Georg Klinikum Eisenach, Klinik f Frauenheilkunde u Geburtshilfe, Eisenach Fig 2.1.17 (A) Moeller H, Berger F Fachärzte f Frauenheilkunde, Lilienthal Fig 2.2.2 (A) Mustea A Universitätsmedizin Greifswald, Klinik Frauenheilkunde und Geburtshilfe, Greifswald Fig 2.1.15 (D); 2.1.19 (C); 2.2.2 (B) Naumann G HELIOS Klinikum Erfurt, Klinik für Frauenheilkunde und Geburtshilfe, Erfurt Fig 2.5.1 (A), (B) Niesel A Klinik Preetz, Gynäkolohie u Entbindung, Preetz Fig 2.1.8 (F) Praski A Marienhaus Klinikum Saarlouis, Klinik für Frauenheilkunde und Geburtshilfe, SaarlouisDillingen Fig 2.1.7 (H) Schwärzler P Asklepios Klinik Barmbek, Gynäkologie und Geburtshilfe, Hamburg Fig 2.1.8 (D), (E) Therre CH Albertinen-Krankenhaus, Klinik f Gynäkologie und Geburtshilfe, Hamburg Fig 2.1.18 (B) Widschwendter P Rotkreuzklinikum München, Gynäkologie, München Fig 2.1.7 (I), (J) Wisch B Regiokliniken, Klinikum Elmshorn, Gynäkologie, Elmshorn Fig 2.2.1 (A), (B) Wojdat R Mathilden Hospital Herford, Klinik Frauenheilkunde und Geburtshilfe, Herford Fig 2.1.14; 2.6.1 (B), (C) Wollensack S Ketteler Krankenhaus, Offenbach, Frauenklinik, Offenbach Fig 2.1.7 (F) Zeisberger, J Kreiskliniken Esslingen, Klinik Nürtingen, Klinik Frauenheilkunde u Geburtshilfe, Nürtingen Fig 2.1.6 (A), (B) Zieger W Schwarzwald-Baar Klinikum, Klinik Frauenheilkunde u Geburtshilfe, VillingenSchwenningen Fig 2.1.7 (D), (E) ... therapy | 47 Aftercare and follow-up, recurrences, metastases and their treatment | 53 Contents | 1. 10 1. 10 .1 1 .10 .2 1. 10.3 1. 11 1 .11 .1 1 .11 .2 1. 11. 3 1. 12 1. 12 .1 1 .12 .2 1. 12.3 1. 13 1. 13 .1 1 .13 .2... radiogenic therapy | X | Contents 1. 4 1. 4 .1 1.4.2 1. 4.3 1. 4.4 1. 5 1. 5 .1 1.5.2 1. 5.3 1. 6 1. 6 .1 1.6.2 1. 6.3 1. 7 1. 7 .1 1.7.2 1. 7.3 1. 8 1. 8 .1 1.8.2 1. 8.3 1. 9 1. 9 .1 1.9.2 1. 9.3 1. 9.4 Cellular leiomyoma | 10 ... 1. 12.3 1. 13 1. 13 .1 1 .13 .2 1. 13.3 1. 13.4 1. 14 1. 14 .1 1 .14 .2 1. 14.3 1. 14.4 1. 15 1. 15 .1 Leiomyoma, its variants and smooth muscle tumors with uncertain malignant potential of the vulva | 56 General,
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