Management of Soft Tissue Sarcoma (eBook)

Murray F. Brennan, MDInternational Center & Department of SurgeryMemorial Sloan-Kettering Cancer CenterNew York, NYCristina R. Antonescu, MDDepartment of PathologyMemorial Sloan-Kettering Cancer CenterNew York, NYKaled M. Alektiar, MDDepartment of Radiation OncologyMemorial Sloan-Kettering Cancer CenterNew York, NYRobert G. Maki, MD, PhDGreat Neck, NYCold Spring Harbor Laboratory Cold Spring Harbor, NY

                                    

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



 

 

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



·         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

                         

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