Sarcoma. A Practical Guide to Multidisciplinary Management

✍ Scribed by Peter F.M. Choong (ed.)

• Publisher: Springer
• Year: 2021
• Tongue: English
• Leaves: 348
• Category: Library

✦ Table of Contents

Preface
About the Editor
Contents
1: Centre-Based Care for Bone and Soft Tissue Sarcoma
1.1 Introduction
1.2 The Rationale for Multidisciplinary Sarcoma Care
1.3 Diagnosis
1.4 Treatment of Bone Cancers
1.5 Treatment of Soft Tissue Sarcoma
1.6 Management of Metastatic Sarcoma
1.7 Multidisciplinary Sarcoma Team and Clinic at St. Vincent’s Hospital Melbourne and Peter MacCallum Cancer Centre
1.7.1 Sarcoma Clinic
1.7.2 Multidisciplinary Meeting
1.7.3 Clinical Trials
1.8 Impact of Delayed Referral on Patient Outcomes
1.9 Recommendation
1.10 Conclusion
References
2: The Epidemiology and Pathogenesis of Sarcoma
2.1 Epidemiology of Sarcomas
2.2 Pathogenesis of Sarcomas
2.2.1 Understanding the Origin of Sarcomas
2.3 Genetic Predisposition Syndromes
2.3.1 Li-Fraumeni Syndrome
2.3.2 Retinoblastoma
2.3.3 Neurofibromatosis
2.3.4 Other Sarcoma-Associated Genetic Syndromes
2.4 Molecular Alterations in Sarcoma
2.4.1 Sarcomas with Simple Karyotypes
2.4.2 Sarcomas with Complex Karyotypes
2.5 Infectious Risk Factors
2.6 Immunological Risk Factors
2.7 Environmental Risk Factors
2.7.1 Radiation Exposure
2.7.2 Chemical Exposure
2.7.3 Trauma Exposure
References
3: Anatomic Imaging of Bone and Soft Tissue Sarcoma
3.1 Plain Radiography
3.2 Ultrasound
3.3 Computed Tomography
3.4 Magnetic Resonance Imaging
3.5 Image Fusion
3.6 The Role of Pre-biopsy Imaging
3.7 Image-Guided Biopsy: Technique
3.7.1 Approach: Broad Principles
3.8 Bone Biopsy
3.9 Soft Tissue Biopsy
3.10 Increasing Role of Interventional Radiology
3.11 Artificial Intelligence
References
4: Nuclear Medicine and Molecular Imaging Techniques
4.1 Bone Scans
4.2 Metabolic Imaging
4.2.1 Thallium-201 Scintigraphy
4.2.2 Tc-99m Pentavalent Dimercaptosuccinic Acid (DMSA(V))
4.2.3 18FDG PET/CT
4.2.3.1 Initial Diagnosis
4.2.3.2 Staging and Restaging
4.2.3.3 Response to Treatment/Prognosis
4.3 Conclusion
References
5: Biopsy
5.1 Background
5.2 Who Should Have a Biopsy?
5.3 Where Should the Biopsy Be Undertaken?
5.4 What Investigations Should Be Undertaken Before Biopsy?
5.5 Who Should Perform the Biopsy?
5.6 Placement of the Biopsy Entry Site (Figs. 5.4, 5.5, and 5.6)
5.7 Biopsy Technique
5.7.1 Open Biopsy
5.7.2 Percutaneous Biopsy
5.7.3 What Tests Should Be Ordered on the Biopsy Material?
5.7.4 How Should the Results of the Biopsy Be Interpreted?
References
6: Bone Tumour Pathology
6.1 Introduction
6.2 Epidemiology
6.3 Classification
6.4 Grading and Staging
6.5 Diagnostic Approach
6.6 Osteogenic Tumours
6.6.1 Benign Osteogenic Lesions
6.6.2 Osteosarcoma
6.6.2.1 Central (Intramedullary) Osteosarcoma
6.6.2.2 Surface Osteosarcoma
6.7 Chondrogenic Tumours
6.7.1 Benign Cartilaginous Tumours
6.7.2 Malignant Cartilaginous Tumours
6.8 Fibrogenic Tumours
6.9 Giant Cell Tumour of the Bone
6.10 Ewing Sarcoma
6.11 Tumours of Haematopoietic Origin
6.11.1 Plasma Cell Myeloma (Multiple Myeloma)
6.11.2 Solitary Plasmacytoma of the Bone
6.11.3 Primary Non-Hodgkin Lymphoma of the Bone
6.12 Langerhans Cell Histiocytosis
References
7: Soft Tissue Tumour Pathology
7.1 General Approach to Soft Tissue Tumours
7.2 Handling Biopsy Specimens
7.3 Classification of Soft Tissue Tumours
7.4 Immunohistochemistry (IHC)
7.4.1 Epithelial Markers
7.4.2 Myogenic Markers
7.4.3 Endothelial (Vascular) Markers
7.4.4 Neural Crest/Melanoma Markers
7.4.5 Other Useful Markers
7.5 Grading Sarcoma
7.6 Problems and Limitations of Grading
7.7 Sarcoma Staging
7.8 Diagnostic Approach to Soft Tissue Tumours with Overlap Patterns
7.9 Spindle Cell Pattern
7.10 Epithelioid Mesenchymal Tumours
7.11 Round Cell Mesenchymal Tumours
7.12 Myxoid Tumours
7.13 Adipocytic Tumours
7.14 Undifferentiated Tumours
References
8: Immunohistochemistry in Bone and Soft Tissue Tumours
8.1 Introduction
8.2 History of IHC: “Putting Tail Lights on Antibodies”
8.3 Procedure, Technical Considerations and Possible Limitations
8.3.1 Preparing the Tissue Sample
8.3.2 Selecting Useful Antibodies
8.3.3 Detecting and Analysing the Reaction
8.4 Immunohistochemical Markers
8.4.1 Broad-Spectrum Markers
8.4.1.1 Cytokeratins
8.4.1.2 Epithelial Membrane Antigen
8.4.1.3 S100 Protein
8.4.1.4 Desmin
8.4.1.5 Smooth Muscle Actin
8.4.1.6 CD34
8.4.1.7 CD99
8.4.2 Novel Markers
8.4.2.1 FLI-1
8.4.2.2 MDM2 and CDK4
8.5 Application and Interpretation of IHC
8.5.1 Identification of Benign Tumours
8.5.1.1 Solitary Fibrous Tumour
8.5.1.2 Nodular Fasciitis
8.5.2 Exclusion of Non-mesenchymal/Non-sarcomatous Tumours
8.5.2.1 Sarcomatoid Carcinoma
8.5.2.2 Malignant Melanoma
8.5.3 For Diagnosis of Mesenchymal Tumours
8.5.3.1 Rhabdomyosarcomas
8.5.3.2 Gastrointestinal Stromal Tumours
8.5.3.3 Malignant Vascular Tumours
References
9: Molecular Genetics in the Multidisciplinary Management of Sarcoma
9.1 Introduction
9.2 Molecular Aberrations in Sarcoma
9.2.1 Chromosomal Translocations
9.2.2 Gene Amplifications
9.2.3 Activating Mutations of Proto-oncogenes
9.2.4 Germline Mutations
9.3 Molecular Techniques
9.3.1 Conventional Karyotyping
9.3.2 Fluorescence In Situ Hybridisation
9.3.3 Reverse Transcription-Polymerase Chain Reaction
9.3.4 Immunohistochemistry
9.4 Diagnosis
9.4.1 Liposarcoma
9.4.2 Synovial Sarcoma
9.4.3 Small Round Cell Tumours
9.4.4 Nodular Fasciitis
9.5 Prognosis
9.6 Treatment
9.6.1 Imatinib in GIST
9.6.2 Imatinib in the Treatment of Other Soft Tissue Tumours
9.6.3 The Future of Other Targeted Therapies in Sarcoma
9.7 Conclusion
References
10: The Role of Radiotherapy for Sarcoma
10.1 Introduction
10.2 The Role of External Beam Radiotherapy in Soft Tissue Sarcoma of Extremity
10.3 Tumour Grade and Radiotherapy
10.4 The Timing of External Beam Radiotherapy
10.5 Target Delineation and Definition of Margins
10.6 Chemoradiotherapy
10.7 Brachytherapy
10.8 The Role of Radiotherapy in Primary Bone Sarcoma
10.9 Osteosarcoma
10.10 Ewing Sarcoma
10.11 Chondrosarcoma
10.12 Whole-Lung Irradiation
10.13 Chordoma
10.14 Retroperitoneal Sarcoma
References
11: The Role of Systemic Therapies in the Management of Soft Tissue Sarcoma
11.1 Introduction
11.2 Systemic Therapy in Localized STS
11.3 Systemic Therapy in Advanced/Metastatic STS
11.4 Traditional Cytotoxic Agents
11.4.1 Doxorubicin and/or Ifosfamide
11.4.2 Other Traditional Cytotoxic Agents
11.4.3 Histotype-Specific Cytotoxic Agents
11.4.3.1 Trabectedin
11.4.3.2 Eribulin
11.4.4 Molecularly Targeted Agents
11.4.5 Tyrosine Kinase Inhibitors (TKIs) or Multi-targeted Kinase Inhibitors
11.4.5.1 Pazopanib
11.5 Treatment Considerations for Certain STS Histology Subtypes
11.5.1 Angiosarcoma
11.5.2 Solitary Fibrous Tumour (SFT)
11.5.3 Perivascular Epithelioid Cell tumour (PEComa)
11.5.4 Alveolar Soft Part Sarcoma (ASPS)
11.6 Treatment Considerations for Certain Benign/Intermediate-Grade Soft Tissue Tumours
11.6.1 Dermatofibrosarcoma Protuberans (DFSP)
11.6.2 Pigmented Villonodular Synovitis (PVNS)
11.6.3 Desmoid-Type Fibromatosis
11.7 Gynaecological Sarcomas
11.7.1 Uterine Sarcoma
11.7.2 Uterine Undifferentiated Pleomorphic Sarcoma (UUPS)
11.7.3 Uterine Leiomyosarcoma (ULMS)
11.7.4 Endometrial Stromal Sarcoma (ESS)
11.7.5 Ovarian Sarcoma
References
12: The Role of Systemic Therapies in the Management of Bone Sarcoma
12.1 Introduction
12.2 Special Considerations
12.2.1 Genetic
12.2.2 Adolescent and Young Adults
12.2.3 Fertility Preservation
12.2.4 Disease and Late Effects Surveillance
12.3 Osteosarcoma
12.3.1 Metastatic Disease
12.3.2 Post-Treatment Surveillance
12.3.3 Recurrent and Relapsed Disease
12.4 Ewing Sarcoma Family of Tumours
12.4.1 Metastatic Disease
12.4.2 Post-Treatment Surveillance
12.4.3 Recurrent and Relapsed Disease
12.4.4 Special Considerations
12.4.4.1 Local Therapy
12.5 Giant Cell Tumour of Bone
12.5.1 Unresectable or Metastatic Disease
12.5.2 Post-Treatment Surveillance
12.6 Chondrosarcoma
12.6.1 Metastatic Disease
12.6.2 Surveillance
12.6.3 Special Consideration
12.6.3.1 Novel Therapies
12.7 Chordoma
12.7.1 Surveillance
12.7.2 Relapsed and Recurrent Disease
References
13: The Importance of Margins in Sarcoma Surgery
13.1 Introduction
13.2 Special Considerations
13.2.1 Response to Neoadjuvant Treatment
13.2.2 Tumour Grade
13.2.3 Unplanned Excision
13.2.4 Histotype and Depth
13.3 MSTS Classification of Surgical Margins
13.3.1 Intralesional Margin
13.3.2 Marginal Margin
13.3.3 Wide Margin
13.3.4 Radical Margin
13.3.5 Adequacy of Surgical Margins
13.3.6 Amputation
References
14: Surgical Management of Lower Limb Sarcomas
14.1 Anatomical Considerations
14.1.1 Soft Tissue Sarcomas
14.1.1.1 Buttock Tumours (Fig. 14.1)
14.1.1.2 Quadriceps Tumours (Fig. 14.2)
14.1.1.3 Adductor Compartment Tumour
14.1.1.4 Hamstring Compartment
14.1.1.5 Popliteal Tumours
14.1.1.6 Calf Tumours
14.1.1.7 Anterior Lower Leg Tumours
14.1.1.8 Foot Tumours
14.1.2 Bone Sarcomas
14.1.2.1 Proximal Femoral Tumours
14.1.2.2 Distal Femoral Tumours
14.1.2.3 Proximal Tibial Tumours
14.1.2.4 Distal Tibial Sarcomas
14.1.3 Investigations
14.1.3.1 Plain Radiographs
14.1.3.2 Anatomic Imaging
14.1.3.3 Functional Imaging
14.1.3.4 Systemic Imaging
14.1.4 Biopsy
14.1.5 Preoperative Preparation
14.1.6 Operative Preparation
14.1.6.1 Anaesthetic
14.1.6.2 Antibiotics
14.1.6.3 Urinary Catheter
14.1.7 Positioning for Soft Tissue Sarcoma Resection
14.1.7.1 Buttock Tumours
14.1.8 Positioning for Bone Sarcoma Resection
14.1.8.1 Proximal and Total Femoral Tumours
14.1.8.2 Knee Tumours
14.1.8.3 Foot Tumours
14.2 Surgical Technique
14.2.1 Key Points for Resection of Soft Tissue Sarcomas
14.2.2 Key Points for Resection of Bone Sarcomas
14.2.2.1 Proximal Femoral Resection
14.2.2.2 Distal Femoral Resection
14.2.2.3 Total Femoral Resection (Fig. 14.8)
14.2.2.4 Proximal Tibial Resection
14.2.2.5 Distal Tibial Resection
14.2.2.6 Amputation
References
15: Surgical Management of Upper Limb Sarcomas
15.1 Introduction
15.2 Soft Tissue Sarcomas
15.2.1 Anatomic Considerations
15.2.2 Shoulder Girdle Tumours
15.2.3 Upper Arm Tumours
15.2.4 Forearm Tumours (Fig. 15.3)
15.2.5 Wrist and Hand Tumours
15.3 Bone Sarcomas
15.3.1 Anatomic Considerations
15.4 Preoperative Staging
15.5 Anatomic Imaging
15.6 Functional Imaging
15.7 Biopsy
15.8 Systemic Imaging
15.9 Surgery
15.9.1 Soft Tissue Sarcomas
15.9.2 Bone Sarcomas
15.9.3 Humerus (Fig. 15.4)
15.9.4 Elbow
15.9.5 Forearm
15.9.6 Amputation
References
16: Surgical Management of Pelvic Sarcomas
16.1 Introduction
16.2 Bone Tumours
16.2.1 Soft Tissue Tumours
16.2.2 Presenting Symptoms and Signs
16.2.3 Investigations
16.2.3.1 Plain Radiography
16.2.3.2 Computed Tomography
16.2.3.3 Magnetic Resonance Imaging
16.2.3.4 Functional Imaging
16.2.3.5 Angiography
16.2.3.6 Ureterogram/Cystogram
16.2.4 Biopsy
16.3 Preoperative Preparation
16.3.1 Positioning
16.3.2 Surgical Considerations
16.3.2.1 Type I Tumours
16.3.2.2 Type II Tumours
16.3.2.3 Type III Tumours
16.3.2.4 Type IV Tumours
Margins
Amputation
References
17: Surgical Management of Chest Wall Sarcomas
17.1 Historical Note
17.2 Primary Chest Wall Malignancies
17.3 Clinical Presentation
17.3.1 Diagnosis
17.3.2 Treatment
17.3.3 Preoperative Evaluation
17.4 Principles of Surgery
17.4.1 Resection
17.4.2 Reconstruction
17.4.3 Choice of Material
17.4.4 Tips/Tricks
17.5 Future Directions
17.6 Summary
References
18: Surgical Management of Pulmonary Metastases from Sarcoma
18.1 Introduction
18.2 The Oligometastatic Disease Concept
18.3 Assessment for Surgery
18.4 Surgical Oncology Principles
18.5 Conduct of Surgery
18.5.1 Access
18.5.2 Wedge Metastasectomy
18.5.3 Anatomic Sublobar Resections
18.5.4 Bronchoplastic Resections
18.6 Complications of Surgery
References
19: Management of Retroperitoneal Sarcomas
19.1 Epidemiology and Staging
19.2 Histologic Subtypes
19.2.1 Liposarcomas
19.2.2 Leiomyosarcomas
19.2.3 Other Histologies
19.3 Patient Presentation
19.4 Diagnostic Pathway
19.4.1 Imaging
19.4.2 Core Needle Biopsy
19.4.3 The Transatlantic Retroperitoneal Sarcoma Working Group
19.5 Management
19.5.1 Multidisciplinary Team
19.5.2 When Is a Retroperitoneal Sarcoma Deemed Unresectable?
19.6 Preoperative Assessment
19.7 The Surgical Strategy
19.8 Prognosis
19.8.1 Neoadjuvant and Adjuvant Therapies
19.8.1.1 Radiotherapy
19.8.1.2 Chemotherapy
19.8.2 Follow-Up
19.9 Management of Recurrent Disease
19.10 Management of Metastatic Disease
References
20: Soft Tissue Reconstructions After Sarcoma Resection
20.1 Challenges of Sarcoma Reconstruction
20.2 Goals and Principles of Reconstruction
20.3 Wound Assessment and Flap Selection
20.4 Regional Reconstruction: Lower Limb
20.4.1 Upper Limb
20.5 Common Flaps
20.6 Propeller Flap or Regional Fasciocutaneous Perforator Flaps
20.6.1 Application in Sarcoma Reconstruction
20.6.2 Latissimus Dorsi Flap
20.6.2.1 History
20.6.3 Application in Sarcoma Reconstruction
20.6.3.1 Free Flap
20.6.3.2 Local Flap
20.6.3.3 Functional Reconstruction
20.6.4 Gracilis Flap
20.6.4.1 History
20.6.4.2 Application in Sarcoma Reconstruction
20.6.5 Parascapular/Scapular Flaps
20.6.5.1 History
20.6.5.2 Application in Sarcoma Reconstruction
20.6.5.3 Local Flap
20.6.5.4 Free Flap
20.6.6 Anterolateral Thigh Flap
20.6.6.1 History
20.6.6.2 Application in Sarcoma Reconstruction
20.6.6.3 Free Flap
20.6.6.4 Pedicle Flap
References
21: Management of Metastatic Sarcoma
21.1 Radiotherapy
21.1.1 Treatment of Oligometastasis
21.1.2 Stereotactic Body Radiotherapy
21.2 Surgery
21.3 Chemotherapy
References