Ebook Sarcoma of the female genitalia (Vol 1): Part 1

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:

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:

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:

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:

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
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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 .
© 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⟩
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♾ Printed on acid-free paper
Printed in Germany
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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 1
Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
1
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
1.1
Angioleiomyoma (Angiomyoma, vascular leiomyoma) | 1
1.1.1
General, epidemiology, etiology, pathogenesis,
pathological-anatomical findings | 1
1.1.2
Clinical presentation, diagnostics, imaging, differential
diagnostics | 3
1.1.3
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 5
1.1.4

Aftercare, recurrences, metastases and their treatment | 6
1.2
Lipoleiomyoma | 6
1.2.1
General, pathogenesis, pathological-anatomical findings | 6
1.2.2
Clinical presentation, diagnostics, imaging, differential
diagnostics | 7
1.2.3
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 8
1.3
Cotyledonoid dissecting leiomyoma | 8
1.3.1
General, pathogenesis, pathological-anatomical findings | 8
1.3.2
Clinical presentation, diagnostics, imaging, differential
diagnostics | 9
1.3.3
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 9


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
General, pathogenesis, pathological-anatomical findings | 10
Clinical presentation, diagnostics, imaging, differential
diagnostics | 13
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 19
Aftercare, recurrences, metastases | 22

Leiomyoma with bizarre nuclei | 23
General, pathogenesis, pathological-anatomical findings | 23
Clinical presentation, diagnostics, imaging, differential
diagnostics | 26
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 26
Mitotically active leiomyoma | 27
General, pathogenesis, pathological-anatomical findings | 27
Clinical presentation, diagnostics, imaging, differential
diagnostics | 28
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 30
Epithelioid leiomyoma | 30
General, pathogenesis, pathological-anatomical findings | 30
Clinical presentation, diagnostics, imaging, differential
diagnostics | 32
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 33
Myxoid leiomyoma | 33
General, pathogenesis, pathological-anatomical findings | 33
Clinical presentation, diagnostics, imaging, differential
diagnostics | 35
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 35
Atypical smooth muscle tumors (smooth muscle tumors with uncertain
malignant potential – STUMP), epithelioid and myxoid leiomyoma with
uncertain malignant potential | 36
General, pathogenesis, pathological-anatomical findings | 36
Clinical presentation, diagnostics, imaging, differential
diagnostics | 43

Course, prognosis, primary surgery, systemic and radiogenic
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
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, pathogenesis, pathological-anatomical findings | 56
Clinical presentation, diagnostics, imaging, differential
diagnostics | 57
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 58
Ovarian and tubal leiomyomas, their variants and smooth muscle
tumors with uncertain malignant potential | 59
General, pathogenesis, pathological-anatomical findings | 59
Clinical presentation, diagnostics, imaging, differential
diagnostics | 60
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 61
Retroperitoneal and vaginal pelvic leiomyomas, their variants and
smooth muscle tumors with uncertain malignant potential | 62
General, pathogenesis, pathological-anatomical findings | 62
Clinical presentation, diagnostics, imaging, differential
diagnostics | 62
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 65
Disseminated (diffuse) peritoneal leiomyomatosis (Leiomyomatosis
peritonealis disseminata) and parasitic leiomyoma | 65
General, pathogenesis, pathological-anatomical findings | 65
Clinical features, diagnostics, imaging, differential diagnostics | 72

Course, prognosis, primary surgery, systemic and radiogenic
therapy | 76
Aftercare, recurrences, metastases, surgical management and
postoperative additive therapy for recurrences and metastatic
disease | 79
Benign metastasizing leiomyoma (MLM) | 81
General, pathogenesis, pathological-anatomical findings | 81
Clinical presentation, diagnostics, imaging, differential
diagnostics | 84
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 88
Aftercare, recurrences, metastases, surgical management and
postoperative additive therapy for recurrences and metastatic
disease | 92
Intravenous (intravascular) leiomyomatosis (IVLM) | 92
General, pathogenesis, pathological-anatomical findings | 92

XI


XII | Contents

1.15.2
1.15.3
1.15.4

Clinical presentation, diagnostics, imaging, differential
diagnostic | 95
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 98

Aftercare, recurrences, metastases, surgical management and
postoperative additive therapy for recurrences and metastatic
disease | 101

Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
2
Leiomyosarcoma | 119
2.1
Uterine leiomyosarcoma | 119
2.1.1
General, epidemiology, etiology, pathogenesis, staging | 119
2.1.2
Macroscopic and microscopic features | 124
2.1.3
Clinical presentation, diagnostics, screening | 134
2.1.4
Imaging | 139
2.1.5
Differential diagnostics | 150
2.1.6
Course, prognosis | 155
2.1.7
Primary surgery | 161
2.1.8
Adjuvant and additive radio-, chemo- and hormone therapy | 166
2.1.9
Primary radio-, chemo- and hormone therapy, approach in cases
of general inoperability | 170
2.1.10
Aftercare, recurrences, metastases | 172

2.1.11
Surgical management and postoperative additive therapy for
recurrences and metastatic disease | 177
2.1.12
Palliative radio-, chemo- and hormone therapy, treatment
with small molecules, supportive therapy | 184
2.2
Leiomyosarcoma of the ovary | 192
2.2.1
General, epidemiology, etiology, pathogenesis, staging,
pathological-anatomical findings | 192
2.2.2
Clinical presentation, diagnostics, imaging, differential
diagnostics | 194
2.2.3
Course, prognosis, primary surgery, adjuvant and additive radioand chemotherapy | 194
2.3
Leiomyosarcoma of the fallopian tube | 196
2.3.1
General, pathogenesis, pathological-anatomical findings | 196
2.3.2
Clinical presentation, diagnostics, imaging, differential
diagnostics | 196
2.3.3
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 196
2.4
Leiomyosarcoma of the uterine, pelvic and ovarian veins | 197
2.4.1
General, pathogenesis, staging, pathological-anatomical

findings | 197


Contents |

2.4.2
2.4.3
2.5
2.5.1
2.5.2
2.5.3
2.6
2.6.1
2.6.2
2.6.3

XIII

Clinical presentation, diagnostics, imaging, differential
diagnostics | 197
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 198
Leiomyosarcoma of the vagina | 199
General, pathogenesis, staging, pathological-anatomical
findings | 199
Clinical presentation, diagnostics, imaging, differential
diagnostics | 200
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 201
Leiomyosarcoma of the vulva | 203

General, pathogenesis, pathologic-anatomical features | 203
Clinical presentation, diagnostics, imaging, differential
diagnostics | 205
Course, prognosis, primary surgery, systemic and radiogenic
therapy | 206

Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
3
Endometrial stromal tumors – endometrial stromal nodule, endometrial
stromal tumor with sex cord-like elements (ESTSCLE), uterine tumor
resembling ovarian sex-cord tumor (UTROSCT) and similar tumors | 231
3.1
Endometrial stromal nodule | 231
3.1.1
Uterine endometrial stromal nodule | 231
3.1.2
Extrauterine endometrial stromal nodules | 243
3.2
Special variants of endometrial stromal tumors | 243
3.2.1
Endometrial stromal tumor with endometrioid glands | 243
3.2.2
Mixed endometrial stromal and smooth muscle tumor,
combined stromal-smooth muscle | 243
3.2.3
Uterine tumor with sex cord-like elements type I
(ESTSCLE, endometrial stromal tumor with sex cord-like
elements) | 244
3.2.4
Uterine tumors with sex cord-like elements type II (UTROSCT, uterine

tumors resembling ovarian sex-cord tumors) | 247
Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
4
Low-grade endometrial stromal sarcoma | 257
4.1
Uterine low-grade endometrial stromal sarcoma | 257
4.1.1
General, epidemiology, etiology, pathogenesis, staging | 257
4.1.2
Macroscopic and microscopic features | 260
4.1.3
Clinical presentation, diagnostics, screening | 267
4.1.4
Imaging | 270


XIV | Contents

4.1.5
4.1.6
4.1.7
4.1.8
4.1.9
4.1.10
4.1.11
4.1.12
4.2

Differential diagnostics | 279
Course, prognosis | 282

Primary surgery | 288
Adjuvant and additive radio-, chemo- and hormone therapy | 291
Primary radio-, chemo- and hormone therapy, approach in cases
of general inoperability | 296
Aftercare, recurrences, metastases | 298
Surgical management and postoperative additive therapy for
recurrences and metastatic disease | 301
Palliative radio-, chemo- and hormone therapy, treatment
with small molecules, supportive therapy | 304
Extrauterine endometrial stromal sarcomas | 309

Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
5
High-grade endometrial stromal sarcoma and undifferentiated uterine
sarcoma | 327
5.1
Uterine high-grade endometrial stromal sarcoma and undifferentiated
uterine sarcoma | 327
5.1.1
General, epidemiology, etiology, pathogenesis, staging | 327
5.1.2
Macroscopic and microscopic features | 329
5.1.3
Clinical presentation, diagnostics, screening | 335
5.1.4
Imaging | 336
5.1.5
Differential diagnostics | 343
5.1.6
Course, prognosis | 346

5.1.7
Primary surgery | 349
5.1.8
Adjuvant and additive radio, chemo and hormone therapy | 351
5.1.9
Primary radio-, chemo- and hormone therapy, approach in cases
of general inoperability | 354
5.1.10
Aftercare, recurrences, metastases | 356
5.1.11
Surgical management and postoperative additive therapy for
recurrences and metastatic disease | 358
5.1.12
Palliative radio-, chemo- and hormone therapy, treatment
with small molecules, supportive therapy | 361
5.2
Extrauterine high-grade endometrial stromal sarcomas
and undifferentiated uterine sarcomas | 365
Günter Köhler, Lars Kaderali, Matthias Evert and Marek Zygmunt
6
Prevention of inadequate sarcoma operations | 373
6.1
Inadequate surgical treatment of uterine sarcomas in leiomyoma
surgery | 373
6.2
Inadequate surgical treatment of sarcomas and STUMP – impact on
prognosis | 373


Contents |

6.3
6.4
6.5
6.6

6.7
6.8

XV

Prevalence of uterine sarcomas in hysterectomies and surgery
performed under the indication of leiomyoma | 380
Anamnestic and clinical criteria suggestive of uterine sarcoma in cases
of supposed leiomyoma | 382
Further, extended diagnostics when there are findings suggestive of
uterine sarcoma in cases of assumed leiomyoma | 388
Diagnostic flowchart for preventing the performance of inadequate
surgery on sarcomas under an assumed indication of
leiomyoma | 395
Risk assessment as a decision-making aid when surgery is planned
under an indication of leiomyoma | 398
Measures to be applied when sarcoma have been subjected
to inadequate surgery under an indication of leiomyoma | 400

Index | 411

Volume 2
Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
1

Angiosarcoma | 1
1.1
Uterine angiosarcoma | 1
1.1.1
General, epidemiology, etiology, pathogenesis, staging | 1
1.1.2
Macroscopic and microscopic features | 2
1.1.3
Clinical presentation, diagnostics, screening | 4
1.1.4
Imaging | 4
1.1.5
Differential diagnostics | 5
1.1.6
Course, prognosis | 6
1.1.7
Primary surgery | 6
1.1.8
Adjuvant and additive radio-, chemo- and hormone therapy | 8
1.1.9
Primary radio-, chemo- and hormone therapy, approach in cases
of general inoperability | 9
1.1.10
Aftercare, recurrences, metastases | 10
1.1.11
Surgery and postoperative additive therapy for recurrences
and metastases | 10
1.1.12
Palliative radio-, chemo- and hormone therapy, small molecule therapy,
supportive therapy | 12

1.2
Extrauterine angiosarcomas | 14
1.2.1
Angiosarcomas of the vulva and the vagina | 14
1.2.2
Angiosarcoma of the ovary and the tuba uterina | 18


XVI | Contents

Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
2
Liposarcoma | 29
2.1
Uterine liposarcoma | 29
2.1.1
General, epidemiology, etiology, pathogenesis, staging | 29
2.1.2
Macroscopic and microscopic features | 31
2.1.3
Clinical presentation, diagnostics, screening | 35
2.1.4
Imaging | 35
2.1.5
Differential diagnostics | 40
2.1.6
Course, prognosis | 40
2.1.7
Primary surgery | 42
2.1.8

Adjuvant and additive radio-, chemo- and hormone therapy | 43
2.1.9
Primary radio-, chemo- and hormone therapy, approach in cases
of general inoperability | 45
2.1.10
Aftercare, recurrences, metastases | 46
2.1.11
Surgical management of and postoperative additive therapy
for recurrences and metastatic disease | 48
2.1.12
Palliative radio-, chemo- and hormone therapy, therapy
with small molecules, supportive therapy | 49
2.2
Extrauterine liposarcomas | 51
2.2.1
Liposarcoma of the vulva and the vagina | 51
2.2.2
Peritoneal liposarcoma | 56
Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
3
Rhabdomyosarcoma | 63
3.1
Uterine rhabdomyosarcoma | 64
3.1.1
General, epidemiology, etiology, pathogenesis, staging | 64
3.1.2
Macroscopic and microscopic features | 70
3.1.3
Clinical presentation, diagnostics, screening | 73
3.1.4

Imaging | 75
3.1.5
Differential diagnostics | 77
3.1.6
Course, prognosis | 78
3.1.7
Primary surgery | 83
3.1.8
Adjuvant and additive radio-, chemo- and hormone therapy | 87
3.1.9
Primary radiation, hormone and chemotherapy, approach in cases
of general inoperability | 91
3.1.10
Aftercare, recurrences, metastases | 94
3.1.11
Surgical management and postoperative additive therapy
for recurrences and metastatic disease | 95
3.1.12
Palliative radiotherapy, chemotherapy and hormone therapy,
therapy with small molecules | 96


Contents |

3.2
3.2.1
3.2.2

XVII

Extrauterine rhabdomyosarcoma | 98
Rhabdomyosarcoma of the ovary and the fallopian tube | 98
Rhabdomyosarcomas of the vulva and the vagina | 102

Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
4
Perivascular epithelioid cell tumor (PEComa) | 117
4.1
PEComa of the uterus | 117
4.1.1
General, epidemiology, etiology, pathogenesis, staging | 117
4.1.2
Macroscopic and microscopic features | 119
4.1.3
Clinical presentation, diagnostics, screening | 122
4.1.4
Imaging | 123
4.1.5
Differential diagnostics | 125
4.1.6
Course, prognosis | 128
4.1.7
Primary surgery | 128
4.1.8
Adjuvant and additive radio-, chemo- and hormone therapy | 129
4.1.9
Primary radio-, chemo- and hormone therapy, approach in cases
of general inoperability | 130
4.1.10
Aftercare, recurrences, metastases | 131

4.1.11
Surgical management and postoperative additive treatment
for recurrences and metastatic disease | 132
4.1.12
Palliative radio-, chemo- and hormone therapy, therapy with small
molecules, supportive therapy | 133
4.2
Extrauterine and extragenital PEComas | 135
4.2.1
General, pathogenesis, pathologic-anatomical features | 135
4.2.2
Clinical presentation, diagnostics, imaging, differential
diagnostics | 136
4.2.3
Course, prognosis, operative, systemic and radiogenic therapy | 138
Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
5
Adenofibroma | 143
5.1
Uterine adenofibroma | 143
5.1.1
General, pathogenesis, pathologico-anatomical features | 143
5.1.2
Clinical presentation, diagnostics, imaging, differential diagnostics,
screening | 146
5.1.3
Course, prognosis, operative, systemic and radiogenic therapy | 148
5.1.4
Aftercare, recurrences, metastases and their treatment | 150
5.2

Extrauterine adenofibromas | 151
5.2.1
Adenofibromas of the ovary and the fallopian tube | 151


XVIII | Contents

Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
6
Adenosarcoma | 157
6.1
Uterine adenosarcoma | 157
6.1.1
General, epidemiology, etiology, pathogenesis, staging | 157
6.1.2
Macroscopic and microscopic features | 159
6.1.3
Clinical presentation, diagnostics, screening | 165
6.1.4
Imaging | 166
6.1.5
Differential diagnostics | 171
6.1.6
Course, prognosis | 173
6.1.7
Primary surgery | 176
6.1.8
Adjuvant and additive radio-, chemo- and hormone therapy | 180
6.1.9
Primary radio-, chemo- and hormone therapy, approach in cases

of general inoperability | 184
6.1.10
Aftercare, recurrences, metastases | 186
6.1.11
Surgical management and postoperative additive therapy
for recurrences and metastatic disease | 188
6.1.12
Palliative radio-, chemo- and hormone therapy,
treatment with small molecules, supportive therapy | 190
6.2
Extrauterine and extragenital adenosarcomas | 194
6.2.1
Adenosarcomas of the ovary and the fallopian tube | 194
6.2.2
Adenosarcoma of the vagina | 196
6.2.3
Extragenital adenosarcomas | 196
Günter Köhler, Katja Evert, Marek Zygmunt and Matthias Evert
7
Carcinosarcoma | 205
7.1
Uterine carcinosarcoma | 205
7.1.1
General, epidemiology, etiology, pathogenesis, staging | 205
7.1.2
Macroscopic and microscopic features | 209
7.1.3
Clinical presentation, diagnostics, screening | 217
7.1.4
Imaging | 219

7.1.5
Differential diagnostics | 226
7.1.6
Course, prognosis | 230
7.1.7
Primary surgery | 234
7.1.8
Adjuvant and additive radio-, chemo- and hormone therapy | 239
7.1.9
Primary radio-, chemo- and hormone therapy, approach in case
of general inoperability | 248
7.1.10
Aftercare, recurrences, metastases | 250
7.1.11
Surgical and postoperative additive treatment for recurrences
and metastatic disease | 252
7.1.12
Palliative radio-, chemo- and hormone therapy,
therapy with small molecules, supportive therapy | 254


Contents |

7.2
7.2.1
7.2.2
7.2.3
7.2.4

XIX

Extrauterine carcinosarcoma | 261
Carcinosarcoma of the ovary | 261
Carcinosarcoma of the fallopian tube | 276
Peritoneal carcinosarcoma | 277
Extraperitoneal carcinosarcoma | 278

Marek Zygmunt, Matthias Evert, Katja Evert and Günter Köhler
8
Fertility and pregnancy and variants of leiomyoma, smooth muscle tumors
of unclear malignant potential, stromal tumors, genital sarcomas, PEComas
and mixed tumors | 297
8.1
General, symptoms, clinical presentation, diagnostics,
differential diagnostics, prognosis | 297
8.2
Disseminated (diffuse) peritoneal leiomyomatosis | 300
8.3
Intravenous (intravascular) leiomyomatosis | 301
8.4
Benign metastasizing leiomyomas | 302
8.5
Leiomyoma and its variants | 303
8.6
Smooth muscle tumors of uncertain malignant potential | 304
8.7
Leiomyosarcoma | 306
8.8
Endometrial stromal nodules and uterine tumors with sex cord-like
elements type I and II (ESTSCLE and UTROSCT) | 308

8.9
Low-grade endometrial stromal sarcoma | 309
8.10
High-grade endometrial stromal sarcomas and undifferentiated
uterine sarcomas | 312
8.11
Angiosarcoma | 313
8.12
Liposarcoma | 313
8.13
Rhabdomyosarcoma | 315
8.14
PEComa | 316
8.15
Adenofibroma | 316
8.16
Adenosarcoma | 317
8.17
Carcinosarcoma | 317
Index | 325



List of abbreviations
AAGL
AB
AF
AI
AJCC
ALM

ANS
AR
ARMS
AS
AUB

American Association of Gynecologic Laparoscopists
Antibody
Adenofibroma
Aromatase inhibitors
American Joint Cancer Committee
Angioleiomyoma
Angiosarcoma
Androgen receptor
Alveolar rhabdomyosarcoma
Adenosarcoma
Abnormal uterine bleeding(s)

BSO
BT

Bilateral Salpingo-oophorectomy
Brachytherapy

CBR
CECT
CHT
CHT-RT
CLM
CR

CS
CT

Clinical benefit rate (CR + PR + SD)
Contrast-enhanced CT
Chemotherapy
Chemoradiotherapy
Cellular leiomyoma
Complete response
Carcinosarcoma
Computed tomography

d
DD
DLM
DFS
DKSM

DNG
DPLM
DSS
DW-MRI

Day
Differential diagnostics
Degenerated leiomyoma
Disease free survival
Deutsches klinisches Kompetenzzentrum für genitale Sarkome und Mischtumoren
Universitätsmedizin Greifswald (German Clinical Center of Excellence for Genital
Sarcomas and Mixed Tumors, University Medicine Greifswald)

Dienogest
Disseminated (diffuse) peritoneal leiomyomatosis
Disease specific survival
Diffusion-weighted magnetic resonance imaging

EGF(R)
EC
ER
ERMS
ERT
ESN
EST
ESTSCLE

Epithelial growth factor (receptor)
Endometrial carcinoma
Estrogen receptor
Embryonal rhabdomyosarcoma
External (percutaneous) radiotherapy
Endometrial stromal nodule
Endometrial stromal tumor
Endometrial stromal tumor with sex cord-like elements


XXII | List of abbreviations

FDG-PET
FES-PET
FS

F-FDG-PET (18 F-2-Fluor-2-deoxy-D-glucose-PET)
F-FES-PET (16α-18 F-fluoro-17β estradiol-PET)
Fibrosarcoma

GCIG
GIST
GnRH

Gynecologic Cancer InterGroup
Gastrointestinal stromal tumors
Gonadotropin-releasing hormone

HE
HE-stain
HG-ESS
HMB45
HPF
HR
HRT
HSC
HT
HU

Hysterectomy
Hematoxylin and eosin stain
High-grade endometrial stromal sarcoma
Human melanoma black 45
High power field (400× magnification)
Hormone receptor
Hormone replacement therapy

Hysteroscopy
Hormone therapy
Hounsfield unit

IHC
IVLM

Immunohistochemistry, immunohistochemical
Intravenous leiomyomatosis

LAVH
LDH
Lig.
LG-ESS
LITT
LLM
LM
L/M-ratio
LMS
LN
LNE
LS
LSC
LVI

Laparoscopically assisted vaginal hysterectomy
Lactate dehydrogenase
Ligamentum, ligament
Low-grade endometrial stromal sarcoma
Laser-induced thermotherapy

Lipoleiomyoma
Leiomyoma
Lymphocyte-monocyte ratio
Leiomyosarcoma
Lymph node
Lymphadenectomy
Liposarcoma
Laparoscopy
Lymphovascular invasion

M/10 HPF
max
MGA
ME
MI
min
MLM
mo
MPA
MRI
MRT
mTOR

Counted mitoses per 10 HPF
Maximal
Megestrol acetate
Myomectomy, myoma enucleation
Mitotic index
Minimal
Benign metastasizing leiomyoma

Months
Medroxyprogesterone acetate
Magnetic resonance imaging
Magnetic resonance tomography
Mammalian target of rapamycin


List of abbreviations

N/L-ratio
NOS
NPV

Neutrophil/lymphocyte ratio
Not otherwise specified
Negative predictive value

PD
PDGF(R)
PEComa
PET-CT
PFI
PFS
PGR
PHH3
PPV
PR
PRMS

Progressive disease

Platelet-derived growth factor (receptors)
Perivascular epithelioid cell tumor
Positron emission tomography
Progression free interval
Progression free survival
Progesterone receptor
Phospho-histone H3
Positive predictive value
Partial response
Pleomorphic rhabdomyosarcoma

q

Time interval

RAH
RFA
RFI
RFS
RI
RMS
RR
RT
RVH

Radical abdominal hysterectomy
Radiofrequency ablation
Relapse free interval
Relapse free survival
Resistance index

Rhabdomyosarcoma
Remission rate
Radiation therapy, radiotherapy, ERT with and without VBT
Radical vaginal hysterectomy

SAH
SD
SEER
SERM
SHE
SI
SIRT
SLH
SLN
SMA
SO
STS
STUMP
SUV

Supracervical abdominal hysterectomy
Stable disease
Surveillance, Epidemiology and End Results
Selective estrogen receptor modulator
Supracervical hysterectomy
Signal intensity (in MRI)
Selective internal radiation therapy
Supracervical laparoscopic hysterectomy
Sentinel lymph node
Smooth muscle actin

Sarcomatous overgrowth
Soft tissue sarcoma
Smooth muscle tumor with uncertain malignant potential
Standardized uptake value

T1W, T2W T1/T2 weighted MRI
T1WC
T1 weighted contrast MRI
T1W-FS
T1 or T2 weighted MRI with fat suppression, occasionally also T1/2W STIR
T2W-FS
see T1W-FS
TAH
Total abdominal hysterectomy
TAS
Transabdominal sonography

|

XXIII


XXIV | List of abbreviations

TCN
THE
TKI
TCS
TLH
TVH

TVS

Tumor cell necrosis
Total hysterectomy
Tyrosine kinase inhibitor
Tubal carcinosarcoma
Total laparoscopic hysterectomy
Total vaginal hysterectomy
Transvaginal sonography

UCS
UES
UPA
UTROSCT
UUS

Uterine carcinosarcoma
Undifferentiated endometrial sarcoma
Ulipristal acetate
Uterine tumor resembling ovarian sex-cord stromal tumor
Undifferentiated uterine sarcoma

VA
VAC
VAI
VBT
VEGF(R)
VI

Vincristine plus actinomycin

Vincristine plus actinomycin D plus cyclophosphamide
Vincristine plus actinomycin D plus ifosfamide
Vaginal brachytherapy
Vascular endothelial growth factor (receptor)
Vascular invasion

wk
WOP
WT-1

Weeks
Week(s) of pregnancy
Wilms tumor Gen 1