The subject matter of volume 2 of the 2-volumes-handbook focusses especially on rare sarcomas of the whole female genitalia. These entities include angiosarcoma, the different lipo- and rhabdomyosarcoma as well as newer entities like the PEComa. Furthermore, mixed mullerian tumors like the benign adenofibroma and the malignant adenosarcoma and carcinosarcoma are described in detail. 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-, radio- and targeted therapy. An own chapter is devoted to the problems of fertility and pregnancy in connection with all sarcomas, variants of leiomyoma, atypical smooth muscle tumors, disseminated peritoneal leiomyomatosis, benign metastasizing leiomyoma, intravenous leiomyomatosis and endometrial stromal tumors - endometrial stromal nodules, endometrial stromal tumor with sex cord-like elements (ESTSCLE), uterine tumor resembling ovarian sex-cord tumor (UTROSCT) -and all mixed tumors - of the female genitalia. The book aims to identify and provide diagnostic and therapeutic guidance. The listed tumor entities also constitute a particular diagnostic challenge for pathologists that contains numerous pitfalls and difficulties. This book, therefore, addresses gynecologists and pathologists in both clinical and private practice, but also surgeons and hemato-oncologists.

• Foreword
• Contents
• List of abbreviations
• Definition of cytoreduction, NCCN-Guidelines
• Immunohistochemistry table
• 1 Angiosarcoma
  • 1.1 Uterine angiosarcoma
    • 1.1.1 General, epidemiology, etiology, pathogenesis, staging
    • 1.1.2 Macroscopic and microscopic features
    • 1.1.3 Clinical presentation, diagnostics, screening
    • 1.1.4 Imaging
    • 1.1.5 Differential diagnostics
    • 1.1.6 Course, prognosis
    • 1.1.7 Primary surgery
    • 1.1.8 Adjuvant and additive radio-, chemo- and hormone therapy
    • 1.1.9 Primary radio-, chemo- and hormone therapy, approach in cases of general inoperability
    • 1.1.10 Aftercare, recurrences, metastases
    • 1.1.11 Surgery and postoperative additive therapy for recurrences and metastases
    • 1.1.12 Palliative radio-, chemo- and hormone therapy, small molecule therapy, supportive therapy
  • 1.2 Extrauterine angiosarcomas
    • 1.2.1 Angiosarcomas of the vulva and the vagina
    • 1.2.2 Angiosarcoma of the ovary and the tuba uterina
• 2 Liposarcoma
  • 2.1 Uterine liposarcoma
    • 2.1.1 General, epidemiology, etiology, pathogenesis, staging
    • 2.1.2 Macroscopic and microscopic features
    • 2.1.3 Clinical presentation, diagnostics, screening
    • 2.1.4 Imaging
    • 2.1.5 Differential diagnostics
    • 2.1.6 Course, prognosis
    • 2.1.7 Primary surgery
    • 2.1.8 Adjuvant and additive radio-, chemo- and hormone therapy
    • 2.1.9 Primary radio-, chemo- and hormone therapy, approach in cases of general inoperability
    • 2.1.10 Aftercare, recurrences, metastases
    • 2.1.11 Surgical management of and postoperative additive therapy for recurrences and metastatic disease
    • 2.1.12 Palliative radio-, chemo- and hormone therapy, therapy with small molecules, supportive therapy
  • 2.2 Extrauterine liposarcomas
    • 2.2.1 Liposarcoma of the vulva and the vagina
    • 2.2.2 Peritoneal liposarcoma
• 3 Rhabdomyosarcoma
  • 3.1 Uterine rhabdomyosarcoma
    • 3.1.1 General, epidemiology, etiology, pathogenesis, staging
    • 3.1.2 Macroscopic and microscopic features
    • 3.1.3 Clinical presentation, diagnostics, screening
    • 3.1.4 Imaging
    • 3.1.5 Differential diagnostics
    • 3.1.6 Course, prognosis
    • 3.1.7 Primary surgery
    • 3.1.8 Adjuvant and additive radio-, chemo- and hormone therapy
    • 3.1.9 Primary radiation, hormone and chemotherapy, approach in cases of general inoperability
    • 3.1.10 Aftercare, recurrences, metastases
    • 3.1.11 Surgical management and postoperative additive therapy for recurrences and metastatic disease
    • 3.1.12 Palliative radiotherapy, chemotherapy and hormone therapy, therapy with small molecules
  • 3.2 Extrauterine rhabdomyosarcoma
    • 3.2.1 Rhabdomyosarcoma of the ovary and the fallopian tube
    • 3.2.2 Rhabdomyosarcomas of the vulva and the vagina
• 4 Perivascular epithelioid cell tumor (PEComa)
  • 4.1 PEComa of the uterus
    • 4.1.1 General, epidemiology, etiology, pathogenesis, staging
    • 4.1.2 Macroscopic and microscopic features
    • 4.1.3 Clinical presentation, diagnostics, screening
    • 4.1.4 Imaging
    • 4.1.5 Differential diagnostics
    • 4.1.6 Course, prognosis
    • 4.1.7 Primary surgery
    • 4.1.8 Adjuvant and additive radio-, chemo- and hormone therapy
    • 4.1.9 Primary radio-, chemo- and hormone therapy, approach in cases of general inoperability
    • 4.1.10 Aftercare, recurrences, metastases
    • 4.1.11 Surgical management and postoperative additive treatment for recurrences and metastatic disease
    • 4.1.12 Palliative radio-, chemo- and hormone therapy, therapy with small molecules, supportive therapy
  • 4.2 Extrauterine and extragenital PEComas
    • 4.2.1 General, pathogenesis, pathologic-anatomical features
    • 4.2.2 Clinical presentation, diagnostics, imaging, differential diagnostics
    • 4.2.3 Course, prognosis, operative, systemic and radiogenic therapy
• 5 Adenofibroma
  • 5.1 Uterine adenofibroma
    • 5.1.1 General, pathogenesis, pathologico-anatomical features
    • 5.1.2 Clinical presentation, diagnostics, imaging, differential diagnostics, screening
    • 5.1.3 Course, prognosis, operative, systemic and radiogenic therapy
    • 5.1.4 Aftercare, recurrences, metastases and their treatment
  • 5.2 Extrauterine adenofibromas
    • 5.2.1 Adenofibromas of the ovary and the fallopian tube
• 6 Adenosarcoma
  • 6.1 Uterine adenosarcoma
    • 6.1.1 General, epidemiology, etiology, pathogenesis, staging
    • 6.1.2 Macroscopic and microscopic features
    • 6.1.3 Clinical presentation, diagnostics, screening
    • 6.1.4 Imaging
    • 6.1.5 Differential diagnostics
    • 6.1.6 Course, prognosis
    • 6.1.7 Primary surgery
    • 6.1.8 Adjuvant and additive radio-, chemo- and hormone therapy
    • 6.1.9 Primary radio-, chemo- and hormone therapy, approach in cases of general inoperability
    • 6.1.10 Aftercare, recurrences, metastases
    • 6.1.11 Surgical management and postoperative additive therapy for recurrences and metastatic disease
    • 6.1.12 Palliative radio-, chemo- and hormone therapy, treatment with small molecules, supportive therapy
  • 6.2 Extrauterine and extragenital adenosarcomas
    • 6.2.1 Adenosarcomas of the ovary and the fallopian tube
    • 6.2.2 Adenosarcoma of the vagina
    • 6.2.3 Extragenital adenosarcomas
• 7 Carcinosarcoma
  • 7.1 Uterine carcinosarcoma
    • 7.1.1 General, epidemiology, etiology, pathogenesis, staging
    • 7.1.2 Macroscopic and microscopic features
    • 7.1.3 Clinical presentation, diagnostics, screening
    • 7.1.4 Imaging
    • 7.1.5 Differential diagnostics
    • 7.1.6 Course, prognosis
    • 7.1.7 Primary surgery
    • 7.1.8 Adjuvant and additive radio-, chemo- and hormone therapy
    • 7.1.9 Primary radio-, chemo- and hormone therapy, approach in case of general inoperability
    • 7.1.10 Aftercare, recurrences, metastases
    • 7.1.11 Surgical and postoperative additive treatment for recurrences and metastatic disease
    • 7.1.12 Palliative radio-, chemo- and hormone therapy, therapy with small molecules, supportive therapy
  • 7.2 Extrauterine carcinosarcoma
    • 7.2.1 Carcinosarcoma of the ovary
    • 7.2.2 Carcinosarcoma of the fallopian tube
    • 7.2.3 Peritoneal carcinosarcoma
    • 7.2.4 Extraperitoneal carcinosarcoma
• 8 Fertility and pregnancy and variants of leiomyoma, smooth muscle tumors of unclear malignant potential, stromal tumors, genital sarcomas, PEComas and mixed tumors
  • 8.1 General, symptoms, clinical presentation, diagnostics, differential diagnostics, prognosis
  • 8.2 Disseminated (diffuse) peritoneal leiomyomatosis
  • 8.3 Intravenous (intravascular) leiomyomatosis
  • 8.4 Benign metastasizing leiomyomas
  • 8.5 Leiomyoma and its variants
  • 8.6 Smooth muscle tumors of uncertain malignant potential
  • 8.7 Leiomyosarcoma
  • 8.8 Endometrial stromal nodules and uterine tumors with sex cord-like elements type I and II (ESTSCLE and UTROSCT)
  • 8.9 Low-grade endometrial stromal sarcoma
  • 8.10 High-grade endometrial stromal sarcomas and undifferentiated uterine sarcomas
  • 8.11 Angiosarcoma
  • 8.12 Liposarcoma
  • 8.13 Rhabdomyosarcoma
  • 8.14 PEComa
  • 8.15 Adenofibroma
  • 8.16 Adenosarcoma
  • 8.17 Carcinosarcoma
• Index