Reed / Siddiqui / Connor Rare and Uncommon Gynecological Cancers

A Clinical Guide
1. Auflage 2010
ISBN: 978-3-642-13492-0
Verlag: Springer
Format: PDF
Kopierschutz: Adobe DRM (»Systemvoraussetzungen)

E-Book, Englisch, 233 Seiten

This book is intended as a reference manual that will provide the busy clinician with up-to-date information on the diagnosis and treatment of uncommon and rare gynecological cancers. While standard textbooks briefly cover these tumors, this is intended as a more comprehensive yet easy-to-use guide. After opening chapters on epidemiology, pathology, and diagnostic imaging, the full range of infrequently encountered gynecological cancers (ovarian, uterine, cervical, vaginal, and vulval) is presented and discussed with the aid of high-quality illustrations. In each case, detailed attention is paid to both differential diagnosis and current treatment options. The book has been written by an international panel of experts and is the first to gather all the uncommon and rare gynecological cancers together within one volume.

Autoren/Hrsg.

Reed, Nicholas

Siddiqui, Nadeem

Connor, Rachel

Inhaltsverzeichnis

1;Contents;5
2;Part I:General Principles;7
2.1;1: Introduction;8
2.1.1;1.1 Rationale for the Textbook;8
2.1.2;1.2 Multidisciplinary Team Management;9
2.1.3;1.3 Structure of the Book;9
2.1.4;1.4 Databases, Registries and Tumour Banks;9
2.1.5;1.5 Clinical Trials;10
2.1.6;1.6 Tumour Sub-Types;10
2.1.7;1.7 Guidelines vs. Protocols;10
2.2;2: Epidemiology and Databases;11
2.2.1;2.1 Rare and Uncommon Gynaecological Cancers;12
2.2.2;2.2 Definition: What is Rare?;12
2.2.3;2.3 Examples of Rare and Uncommon Cancers;13
2.3;3: Rare and Uncommon Gynaecological Cancers: A Clinical Guide;14
2.3.1;3.1 Pathology;14
2.3.2;References;16
2.4;4: The Contribution of Diagnostic Imaging in Rare Gynaecological Malignancies;18
2.4.1;4.1 Introduction;18
2.4.2;4.2 Imaging Techniques for Evaluating the Primary Gynaecological Malignancy;18
2.4.2.1;4.2.1 Ultrasound;18
2.4.2.2;4.2.2 CT;19
2.4.2.3;4.2.3 CT/PET;19
2.4.2.4;4.2.4 MRI;20
2.4.3;4.3 Lymph Node Imaging in Gynaecological Malignancy;23
2.4.4;4.4 Staging Considerations;24
2.4.4.1;4.4.1 Uterus;24
2.4.4.2;4.4.2 Cervix;26
2.4.4.3;4.4.3 Vagina;28
2.4.4.4;4.4.4 Vulva;28
2.4.4.5;4.4.5 Ovary;29
2.4.5;4.5 Imaging Characteristics of Specific Tumours;30
2.4.5.1;4.5.1 Uterus;30
2.4.5.1.1;4.5.1.1 Clear Cell and Serous Endometrial Tumours;30
2.4.5.1.2;4.5.1.2 Carcinosarcoma;31
2.4.5.1.3;4.5.1.3 Leiomyosarcoma and Endometrial Stromal Sarcoma;33
2.4.5.1.4;4.5.1.4 Leiomyosarcoma;37
2.4.5.1.5;4.5.1.5 Adenosarcoma;41
2.4.5.2;4.5.2 Cervical Cancers;41
2.4.5.2.1;4.5.2.1 Small Cell Carcinoma;43
2.4.5.3;4.5.3 Uterine and Cervical lymphoma;44
2.4.5.4;4.5.4 Cervical Sarcomas;45
2.4.5.5;4.5.5 Vaginal Tumours;45
2.4.5.6;4.5.6 Rare Ovarian Tumours;47
2.4.5.7;4.5.7 Germ Cell Tumours;48
2.4.5.8;4.5.8 Teratoma;48
2.4.5.9;4.5.9 Carcinoid Tumours;49
2.4.5.10;4.5.10 Immature Teratomas;50
2.4.5.11;4.5.11 Yolk Sac Tumours;53
2.4.5.12;4.5.12 Sex Cord Stromal Tumours;53
2.4.5.13;4.5.13 Granulosa Tumours;53
2.4.5.14;4.5.14 Steroid Cell Tumours;56
2.4.5.15;4.5.15 Stromal Tumours;57
2.4.5.16;4.5.16 Rare Epithelial Tumours;58
2.4.5.16.1;4.5.16.1 Clear Cell Carcinoma;58
2.4.5.17;4.5.17 Brenner Tumours;59
2.4.5.18;4.5.18 Small Cell Ovarian Cancer;61
2.4.5.19;4.5.19 Fallopian Tube Carcinoma;63
2.4.6;References;65
3;Part II:Ovarian Rare Cancers;68
3.1;5: Mucinous Cancers: Ovary;69
3.1.1;5.1 Introduction and Epidemiology;69
3.1.2;5.2 Pathology;69
3.1.3;5.3 Biomarkers and Immunophenotyping;70
3.1.4;5.4 Clinical Features: Primary vs. Secondary Cancer;70
3.1.5;5.5 Clinical Management;71
3.1.5.1;5.5.1 Imaging;71
3.1.6;5.6 Tumour Markers;71
3.1.7;5.7 Endoscopy/Colonoscopy;71
3.1.8;5.8 Treatment of Mucinous Epithelial Ovarian Cancer;71
3.1.9;5.9 Treatment of Advanced Mucinous Ovarian Cancer;72
3.1.10;5.10 New Studies for Mucinous Ovarian Cancer;73
3.1.11;References;74
3.2;6: Pseudomyxoma Peritonei;76
3.2.1;6.1 Introduction;76
3.2.2;6.2 Origin of PMP;76
3.2.3;6.3 Pathophysiology of PMP;77
3.2.3.1;6.3.1 The Redistribution Phenomenon;77
3.2.4;6.4 Pathological Classification;78
3.2.5;6.5 Diagnosis;78
3.2.5.1;6.5.1 Preop Assessment: Tumour Markers – CEA, CA125, CA19.9 and Laparoscopy;79
3.2.5.2;6.5.2 Operative Strategy in Unexpected Case;79
3.2.6;6.6 Treatment;79
3.2.6.1;6.6.1 Surgical Treatment of PMP;79
3.2.6.2;6.6.2 Rationale for Hyperthermic Intraperitoneal Chemotherapy (HIPEC);79
3.2.6.3;6.6.3 Survival Following CRS and HIPEC;80
3.2.6.4;6.6.4 Morbidity and Mortality of CRS with HIPEC;80
3.2.7;6.7 Conclusion;80
3.2.8;References;80
3.3;7: Ovarian Clear Cell Carcinoma;83
3.3.1;7.1 Introduction;83
3.3.2;7.2 Management;84
3.3.3;7.3 Histopathology and Molecular Characterisation;85
3.3.4;7.4 Biomarkers;86
3.3.5;7.5 Therapeutic Targets;86
3.3.6;References;88
3.4;8: Clear Cell Carcinoma of the Ovary;91
3.4.1;8.1 Incidence and Clinical Behavior;91
3.4.2;8.2 Histopathological Issues;91
3.4.2.1;8.2.1 Histopathological Characteristics;91
3.4.2.2;8.2.2 Origin of CCC;92
3.4.3;8.3 Molecular Biology;93
3.4.4;8.4 Surgery and Surgical Staging;94
3.4.4.1;8.4.1 Cytoreductive Surgery;94
3.4.4.2;8.4.2 Lymph-Node Metastasis;94
3.4.4.3;8.4.3 Stage Distribution;95
3.4.5;8.5 Adjuvant Treatment;95
3.4.5.1;8.5.1 Response Rate in Postoperative Chemotherapy (Table 8.2);95
3.4.5.1.1;8.5.1.1 Conventional Platinum-Based Chemotherapy;95
3.4.5.1.2;8.5.1.2 Combination Therapy of Paclitaxel and Platinum;95
3.4.5.2;8.5.2 Survival Rate and Time;95
3.4.5.2.1;8.5.2.1 Conventional Platinum-Based Chemotherapy;95
3.4.5.2.2;8.5.2.2 Combination Therapy of Paclitaxel and Platinum;96
3.4.5.2.3;8.5.2.3 Combination Therapy with Irinotecan;97
3.4.6;8.6 Mechanisms of Chemotherapy Resistance (Table 8.3);98
3.4.6.1;8.6.1 ABC Transporter;98
3.4.6.2;8.6.2 Drug Inactivation;98
3.4.6.3;8.6.3 Low Cell Proliferation;98
3.4.6.4;8.6.4 Stress Response, Glycogenesis and Glycolysis;98
3.4.6.5;8.6.5 Other;99
3.4.7;8.7 Future Therapy;99
3.4.8;References;100
3.5;9: The Continuum of Serous Ovarian Tumors of Low Malignant Potential and Low-Grade Serous Carcinoma of the Ovary;104
3.5.1;9.1 Introduction;104
3.5.2;9.2 Serous Tumors of Low Malignant Potential;104
3.5.3;9.3 Low-Grade Serous Carcinoma;105
3.5.3.1;9.3.1 Pathology;106
3.5.3.2;9.3.2 Clinical Behavior and Management;107
3.5.3.3;9.3.3 Molecular Biology;108
3.5.3.3.1;9.3.3.1 Mutational Analyses;108
3.5.3.3.2;9.3.3.2 Expression Profiling Studies;108
3.5.3.3.3;9.3.3.3 Other Biomarkers;109
3.5.4;9.4 Summary;109
3.5.5;References;109
3.6;10: Sex Cord-Stromal Tumors;111
3.6.1;10.1 Ovarian Sex Cord-Stromal Tumors;111
3.6.1.1;10.1.1 Overview and Epidemiology;111
3.6.1.2;10.1.2 Pathology, Molecular Characteristics, and Associated Biomarkers;113
3.6.1.2.1;10.1.2.1 Pathology;113
3.6.1.2.2;10.1.2.2 Molecular Characteristics;115
3.6.1.2.3;10.1.2.3 Associated Biomarkers;116
3.6.1.3;10.1.3 Diagnosis;116
3.6.1.4;10.1.4 Imaging;117
3.6.1.5;10.1.5 Surgical Therapy and Staging;118
3.6.1.5.1;10.1.5.1 General Treatment Guidelines: Surgical Therapy;118
3.6.1.6;10.1.6 Adjuvant Therapy;120
3.6.1.7;10.1.7 Treatment for Recurrent Disease;122
3.6.2;10.2 Uterine Tumors Resembling Ovarian Sex Cord-Stromal Tumors;123
3.6.3;References;124
3.7;11: Squamous Cell Carcinomas Arising From Dermoids;129
3.7.1;References;131
3.8;12: Ovarian Carcinosarcomas;132
3.8.1;12.1 Incidence and Epidemiology;132
3.8.2;12.2 Pathology;133
3.8.3;12.3 Investigations and Initial Management;133
3.8.4;12.4 Surgical Management;133
3.8.5;12.5 Post-Operative Management;134
3.8.6;12.6 Modern Approach to Management;135
3.8.7;12.7 The Future;136
3.8.8;12.8 Conclusions;137
3.8.9;References;137
3.9;13: Small Cell and Neuroendocrine Cancers of the Ovary;140
3.9.1;13.1 Introduction;140
3.9.2;13.2 Pathology;140
3.9.3;13.3 Clinical Presentation;141
3.9.4;13.4 Post-Operative Management;142
3.9.5;13.5 Recent Literature;142
3.9.6;References;143
3.10;14: Primary Ovarian Carcinoids and Neuro-Endocrine Tumours Including Struma Ovarii;145
3.10.1;14.1 Primary Insular Carcinoids of the Ovary;145
3.10.2;14.2 Primary Trabecular Carcinoids of the Ovary;145
3.10.3;14.3 Primary Mucinous Ovarian Carcinoids;146
3.10.4;14.4 Strumal Carcinoids;146
3.10.5;14.5 Metastases;146
3.10.6;14.6 Pathology;146
3.10.7;14.7 Carcinoid Syndrome;146
3.10.8;14.8 Imaging;147
3.10.9;14.9 Biochemical and Tumour Markers;147
3.10.10;14.10 Management and Clinical Course;147
3.10.11;14.11 Treatment of Relapsed Disease;148
3.10.12;14.12 Treatment of Ovarian Strumal Carcinoids;149
3.10.13;References;149
4;Part III:Uterine Rare Cancers;151
4.1;15: Reed Uterine Carcinosarcomas;152
4.1.1;15.1 Introduction;152
4.1.2;15.2 Presentation;152
4.1.3;15.3 Staging Investigations;153
4.1.4;15.4 Pathology;154
4.1.5;15.5 Surgical Management;154
4.1.6;15.6 Postoperative Adjuvant Therapies;155
4.1.6.1;15.6.1 To Irradiate or Not?;155
4.1.6.2;15.6.2 Adjuvant Hormonal Therapy;156
4.1.6.3;15.6.3 Adjuvant Chemotherapy;156
4.1.7;15.7 Recommendations of Care;157
4.1.8;15.8 Recurrent and Advanced Disease;157
4.1.9;15.9 Conclusions;160
4.1.10;References;160
4.2;16: Leiomyosarcomas of Uterus;163
4.2.1;16.1 Introduction;163
4.2.2;16.2 Incidence and Epidemiology;163
4.2.3;16.3 Presentation;164
4.2.4;16.4 Pathology;165
4.2.5;16.5 Surgical Management;165
4.2.6;16.6 Post-Operative Care;166
4.2.6.1;16.6.1 Adjuvant Treatments;166
4.2.6.2;16.6.2 Radiation Therapy;166
4.2.6.3;16.6.3 Adjuvant Chemotherapy;167
4.2.7;16.7 Recurrent and Advanced Disease;168
4.2.8;16.8 Recurrent or Locally Advanced Leiomyosarcomas;168
4.2.8.1;16.8.1 Hormonal Therapies;169
4.2.8.2;16.8.2 Chemotherapy;169
4.2.8.3;16.8.3 Combination Therapy;170
4.2.8.4;16.8.4 Targeted Agents;170
4.2.9;16.9 Lung Metastases;171
4.2.10;16.10 Clinical Trials;171
4.2.11;16.11 Conclusions;171
4.2.12;References;171
4.3;17: Mucinous Tumours of the Uterine Corpus;174
4.3.1;17.1 Presentation;174
4.3.2;17.2 Surgical Management;174
4.3.3;17.3 Post-Operative Management;174
4.3.4;References;175
4.4;18: Clear Cell Cancers of Uterus;176
4.4.1;18.1 Incidence and Epidemiology;176
4.4.2;18.2 Clinical Presentation;176
4.4.3;18.3 Imaging and Diagnostic Work Up;176
4.4.4;18.4 Pathology;176
4.4.5;18.5 Surgical Assessment and Management;177
4.4.6;18.6 Adjuvant Post-Operative Treatments;178
4.4.6.1;18.6.1 External Beam Radiation Therapy;179
4.4.6.2;18.6.2 Brachytherapy;179
4.4.6.3;18.6.3 Chemo-Radiotherapy;180
4.4.7;18.7 Adjuvant Chemotherapy;180
4.4.7.1;18.7.1 Relapsed/Advanced Local Disease;180
4.4.8;18.8 Follow-Up Protocols;181
4.4.9;18.9 Relapsed Disease;181
4.4.10;References;181
5;Part IV:Cervix and Vulval Cancers;185
5.1;19: Small Cell and Neuroendocrine Cancers of the Cervix;186
5.1.1;19.1 Introduction;186
5.1.2;19.2 Presentation;187
5.1.3;19.3 Pathology;187
5.1.4;19.4 Staging;188
5.1.5;19.5 Treatment Options;188
5.1.6;19.6 Localised Disease;188
5.1.6.1;19.6.1 The Role of Surgery;188
5.1.6.2;19.6.2 Adjuvant Therapy;189
5.1.6.3;19.6.3 Primary Chemo-Radiation;189
5.1.6.4;19.6.4 Multi-Modality Therapy;189
5.1.7;19.7 Extensive Disease;190
5.1.8;19.8 Large Cell Variant of Small Cell Cancer;190
5.1.9;19.9 Conclusions;190
5.1.10;References;191
5.2;20: Primary Malignant Melanoma of the Vulva and Vagina;193
5.2.1;20.1 Introduction;193
5.2.2;20.2 Demographics and Aetiology;193
5.2.3;20.3 Clinical Presentation;193
5.2.4;20.4 Histopathological Subtypes;193
5.2.5;20.5 Staging and Prognosis;194
5.2.6;20.6 Role of Primary Surgery;195
5.2.7;20.7 Assessment of Lymph Node Status;195
5.2.8;20.8 Role of Adjuvant Therapy;196
5.2.9;20.9 Conclusion;196
5.2.10;References;196
5.3;21: Gynecologic Cancers in Pregnancy: Guidelines of an International Consensus Meeting;198
5.3.1;21.1 Introduction;198
5.3.2;21.2 Imaging and Oncological Treatment Modalities During Pregnancy;199
5.3.2.1;21.2.1 Imaging and Diagnosis During Pregnancy;200
5.3.2.2;21.2.2 Surgery During Pregnancy;201
5.3.2.3;21.2.3 Systemic Anticancer Treatment During Pregnancy;201
5.3.2.4;21.2.4 Radiotherapy During Pregnancy;203
5.3.2.5;21.2.5 Supportive Therapy and Symptom Control in the Pregnant Patient;204
5.3.3;21.3 Monitoring Pregnancy and Neonatal Outcome;204
5.3.3.1;21.3.1 Monitoring of the Pregnancy, Complicated with a Gynecologic Cancer;204
5.3.3.2;21.3.2 Neonatal and Long-Term Outcome After In Utero Exposure to Chemotherapy;205
5.3.4;21.4 Organ Pathology;206
5.3.4.1;21.4.1 Pre-Invasive Cervical Cancer;206
5.3.4.2;21.4.2 Invasive Cervical Cancer;206
5.3.4.3;21.4.3 Vulvar Cancer;208
5.3.4.4;21.4.4 Endometrial Cancer;209
5.3.4.5;21.4.5 Ovarian Neoplasm;209
5.3.4.6;21.4.6 Psychosocial and Ethical Concerns of Cancer Diagnosis During Pregnancy;210
5.3.5;21.5 Conclusion;211
5.3.6;References;211
6;Index;217