Management of Soft Tissue Sarcoma
Table of Contents
Preface
Part I Introduction
1. General Description
· Introduction
· Incidence and Prevalence
· Predisposing and Genetic Factors
· References
2. Natural History - Importance of Size, Site, Histopathology
· Natural History
· Influence of Site
· Staging
· Staging of Retroperitoneal Visceral Sarcoma
· Prognostic Factors for Extremity and Superficial Soft Tissue Sarcoma
· Disease-Specific Survival
· Prognostic Factors for Survival Following Local Recurrence of Extremity Sarcoma
· Prognostic Factors: Nomograms
· References
3. General Statement as to Efficacy Surgery / Chemotherapy / Radiation Therapy
· Extent of primary surgery
· Surgical Treatment of Local Recurrence
· Diagnostic Imaging
· Surgery for Metastatic Disease
o Pulmonary Metastasis
o Surgery and the Management of Sarcoma Liver Metastasis
· Radiation Therapy
o Adjuvant Radiation Therapy
o Dose of Radiation Therapy
o Adjuvant Brachytherapy
o Radiation Therapy in the Presence of Positive Microscopic Margins
o Definitive Radiation Therapy
o Radiation to Patients with Neurovascular Involvement
o Wound Complications of Radiation Therapy
o Adjuvant and Neoadjuvant Chemotherapy for Soft Tissue Sarcomas
o Sarcomas More Common in Adults
o Larger Randomized Studies
o Selected Meta-analyses of Randomized Trials of Adjuvant Chemotherapy
o Adjuvant Therapy for GIST
o Sarcomas More Common in Pediatric Setting
o Brief comments regarding chemotherapy for metastatic soft tissue sarcoma
o Newer Systemic Agents
· Special Techniques for Primary & Locally Recurrent Disease
o Intra-arterial Chemotherapy
o Limb Perfusion and Hyperthermia
o Immunotherapy for Sarcomas
<· References
Part II Management by Histopathology
4. Gastrointestinal stromal tumor
· Imaging
· Familial GIST
· Natural History
· Diagnosis / Molecular Pathology
· Treatment
o Adjuvant Imatinib for Primary GIST
o Neoadjuvant Therapy for Primary Disease not amenable to Surgery<
o Treatment of Recurrence
o Imatinib: The First Small Molecule Oral Kinase Inhibitor for Metastatic GIST Failing Imatinib & Sunitinib
o Systemic Targeted Therapy for Metastatic GIST
o Dose Intensity over Time
o Imatinib Pharmacokinetics
· Second-Line Sunitinib for Imatinib-Resistant Metastatic GIST
o Other Tyrosine Kinase Inhibitors for Metastatic GIST
o Failing Imatinib and Sunitinib
o GRID Trial
· Newer Agents for GIST
· References
5. Liposarcoma
· Imaging
· Diagnosis
· Treatment
· Radiation Therapy for Liposarcoma
· Systemic Therapy: General Considerations
· Adjuvant Therapy
· Treatment of Metastatic Disease
· Outcome
· Outcome Following Metastasis
· References
6. Leiomyosarcoma
· Imaging
· Diagnosis, Molecular Pathology
· Primary Treatment
· Radiation Therapy
· Systemic Therapy
o Adjuvant Chemotherapy for Leiomyosarcoma
· Outcomes after Primary Therapy
· Patterns of Recurrence
· Treatment of Recurrence
· Metastatic Disease
· References
7. Undifferentiated Pleomorphic Sarcoma (UPS) (Malignant Fibrous Histiocytoma: MFH) Myxofibrosarcoma
· Imaging
· Diagnosis, Molecular Pathology
· Natural History
· Treatment
· Radiation Therapy
· Metastatic Disease
· Adjuvant Chemotherapy
· Outcome
· References
8. Synovial Sarcoma
· Imaging
· Diagnosis, Molecular Pathology
· Treatment
· Radiation Therapy
· Chemotherapy
· Treatment of Recurrence
o Local Recurrence
o Systemic Treatment
· Outcome
· References
9. Malignant Peripheral Nerve Sheath Tumor (MPNST) / Triton Tumor
· Presentation
· Imaging
· Diagnosis, Pathology
· Neurofibromatosis Type 1 and Outcome
· Treatment
· Radiation Therapy
· Chemotherapy for MPNST
· Outcome
· References
10. Desmoid Tumor / Deep-Seated Fibromatosis (Desmoid-Type Fibromatosis)
· Clinical Presentation
· Imaging
· Diagnosis, Molecular Pathology
· Natural History
· Treatment
· Treatment of Recurrence
<· Systemic Therapy
· Treatment by Observation
· Radiation Therapy
· Patterns of Failure
· Outcome
· References
11. Solitary Fibrous Tumor / Hemangiopericytoma
· Doege-Potter Syndrome
· Primary Therapy
· Systemic Therapy for Metastatic Disease
· Outcome
· References
12. Fibrosarcoma and its Variants
· Outcome
· Dermatofibrosarcoma Protuberans
· Outcome
· Low-Grade Fibromyxoid Sarcoma (of Evans Tumor)
· Outcome
· Sclerosing Epithelioid Fibrosarcoma
· Inflammatory Myofibroblastic Tumor
· Infantile Fibrosarcoma
· Myxoinflammatory Fibroblastic Sarcoma / Inflammatory Myxohyaline Tumor of Distal Extremities
· Adult-Type Fibrosarcoma
· References
13. Vascular Sarcomas
· Epithelioid Hemangioendothelioma
· Angiosarcoma / Lymphangiosarcoma
· Outcome
· Kaposi Sarcoma
· References
14. Epithelioid Sarcoma
· Outcome
· References
15. Sarcomas More Common in Children
Soft Tissue Sarcomas More Commonly Observed in Pediatric Patients - Ewing Sarcoma Family of Tumors (EFT)
- Demographics
- Primary Therapy
- Adjuvant Chemotherapy
- High-Dose Systemic Therapy for Metastatic Disease
- Standard Cytotoxic Chemotherapy After Disease Relapse
- Investigational Approaches
- Ewing Sarcoma-Like Tumors
- Rhabdomyosarcoma
- Demographics
- Molecular Biology
- Risk Stratification
- Staging
- Imaging
Primary Therapy - Chemotherapy for Metastatic Disease
- Embryonal Sarcoma
- References
16. Radiation-Induced Sarcoma
· References
17. Alveolar Soft Part Sarcoma
· Imaging
· Diagnosis, Molecular Pathology
· Primary Treatment
· Treatment of Metastatic Disease
· Outcome
· References
18. Clear Cell Sarcoma / Melanoma of Soft Parts
· Imaging
· Diagnosis, Molecular Pathology
· Treatment
· Outcome
·
19. Desmoplastic Small Round Cell Tumor
· Imaging
· Diagnosis
· Treatment
· Outcome
· References
20. Extraskeletal Myxoid Chondrosarcoma
· Imaging
· Diagnosis
· Treatment
· Outcome
· Mesenchymal Chondrosarcoma
· References
21. Other Uterine Sarcomas
· Low Grade Endometrial Stromal Sarcoma
o Diagnosis
o Treatment
o Outcome
· High Grade Endometrial Stromal Sarcoma
o Outcome
· Undifferentiated Uterine Sarcoma (UUS)
· Uterine Carcinosarcomas and Other Malignant Mixed Müllerian Tumors
· PEComas
o Outcome
· References
22. Extraskeletal Osteogenic Sarcoma
· Imaging
· Diagnosis
· Treatment
· Outcome
· References
23. Sustentacular Tumors of Lymph Tissue
· Follicular Dendritic Cell Tumor (Dendritic Reticulum Cell Tumor [FDCT]) and Interdigitating Reticulum Cell Tumor (IDRCT)
· True Histiocytic Sarcoma
· Langerhans Cell Tumors
· Outcome
· References
24. Uncommon / Unique Sites
· Heart and Great Vessels
· Primary Sarcomas of the Breast
o Phylloides Tumor
o Diagnosis
o Outcome
· Head and Neck
o Treatment
· Primary Sarcomas of the Mediastinum
· Liver
·
Part III Benign and Less Aggressive Lesions
25. Mostly Benign / Rarely Metastasizing
· Ossifying Fibromyxoid Tumor
· Perivascular Epithelial Cell Tumor (PEComa) & Related Entities, Lymphangioleiomyomatosis, Angiomyolipoma, and Sugar Cell Tumor
· Therapy
· Giant Cell Tumor of Tendon Sheath / Pigmented Villonodular Synovitis
· Myoepithelioma of Soft Tissue
· Glomus tumor <· References
26. Benign Tumors
· Lipoma
· Lipomatosis
· Lipoblastoma / Lipoblastomatosis
· Angiolipoma
· Angiomyolipoma
· Angiomyelolipoma
· Hibernoma
· Elastofibroma
· Granular Cell Tumor
· Hemangioma
· Leiomyoma
· Schwannoma
· Neurofibroma
· Myxoma
· Angiomyxoma
· Angiofibroma
· References
27. Reactive Lesions
· Myositis Ossificans
· Nodular Fasciitis
· Sarcoma Masquerade
· References
Suggested Keywords
Index