Pediatric bone and soft tissue sarcomas:
Gespeichert in:
| Weitere Verfasser: | |
|---|---|
| Format: | Elektronisch E-Book |
| Sprache: | German |
| Veröffentlicht: |
Berlin [u.a.]
Springer
2006
|
| Schriftenreihe: | Pediatric oncology
|
| Schlagworte: | |
| Online-Zugang: | Volltext Inhaltsverzeichnis |
| Beschreibung: | Literaturangaben |
| Beschreibung: | 1 Online-Ressource (XIV, 240 S.) |
| ISBN: | 9783540294474 |
| DOI: | 10.1007/3-540-29447-3 |
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| adam_text | 1 Epidemiology of Bone
and Soft Tissue Sarcomas
Logan G. Spector, Julie A. Ross,
Rajaram Nagarajan
1.1 Descriptive Epidemiology of Bone Tumors. ... 1
1.2 Worldwide Statistics 1
1.3 North American Statistics 3
1.4 Mortality and Survivorship 4
1.5 Etiology of Bone Sarcomas 5
1.6 Bone Sarcomas: Future Directions 7
1.7 Epidemiology of Soft Tissue Sarcomas 7
References 9
2 Pathologic and Molecular Techniques
Used in the Diagnosis
and Treatment Planning of Sarcomas
Timothy Triche, Deborah Schofield
2.1 Introduction 13
2.2 Standard Histopathology 14
2.2.1 Light Microscopy 14
2.2.2 Immunohistochemistry 15
2.2.3 Electron Microscopy 16
2.3 Cytogenetics 16
2.3.1 Classical Cytogenetic Analysis 16
2.3.2 In Situ Hybridization 18
2.3.3 Spectral Karyotyping 19
2.3.4 Comparative Genomic Hybridization .... 20
2.4 Molecular Genetic Techniques 20
2.4.1 Introduction 20
2.4.2 PCRandQ PCR 20
2.4.3 Microarrays 23
2.4.4 Proteomics 27
2.4.5 Conclusion 30
References 31
I
3 Diagnostic Imaging of Pediatric Bone
and Soft Tissue Sarcomas
Thomas D. Henry, Mary E. McCarville,
Fredric A. Hoffer
3.1 Introduction 35
3.2 Initial Evaluation of Bone
and Soft Tissue Sarcomas 35
3.3 Imaging Techniques
for Evaluating the Primary Tumor 37
3.4 Staging 38
3.4.1 Local Extent of the Tumor 38
3.4.2 Detection of Metastatic Disease 40
3.5 Criteria for Evaluating Treatment Response ... 41
3.6 Outcome Criteria 43
3.7 Biopsy of the Primary Lesion 43
3.8 Imaging Characteristics of Specific Tumors ... 44
3.8.1 Bone Sarcomas 44
3.8.1.1 Osteosarcoma 44
3.8.1.2 Chondrosarcoma 48
3.8.1.3 Ewing Sarcoma of Bone 48
3.8.2 Soft Tissue Sarcomas 51
3.8.2.1 Differentiating Benign
from Malignant Soft Tissue Lesions 51
3.8.2.2 Soft Tissue Ewing Sarcoma Family
ofTumors 52
3.8.2.3 Rhabdomyosarcoma 54
3.8.2.4 Non rhabdomyosarcoma
Soft Tissue Sarcomas 61
3.8.2.4.1 Synovial Sarcoma 61
3.8.2.4.2 Congenital Fibrosarcoma 62
3.8.2.4.3 Hemangiopericytoma 63
3.8.2.4.4 Malignant Peripheral Nerve
Sheath Tumors IMPNST) 65
3.9 Post treatment Imaging Concerns 66
3.10 Radiofrequency Ablation 66
References 67
m
4 Local Control Issues in Pediatric Bone
and Soft Tissue Sarcomas
John C. Breneman, David Rodeberg,
Ruth F. Lavigne, Ken Brown, Eugene S. Wiener
4.1 Significance of Local Control
in Pediatric Sarcomas 71
4.2 Background to Local Control Modalities 72
4.2.1 Patterns of Tumor Growth 72
4.2.2 Surgery 72
4.2.2.1 Biopsy Guidelines 72
4.2.2.2 Surgical Margins 72
4.2.3 Radiation Therapy ... 73
4.2.3.1 Types of Radiation 73
4.2.3.2 Techniques of Radiotherapy .... 73
4.2.3.3 Treatment Morbidity 74
4.2.4 Combined Surgery and Radiotherapy
for Local Control 74
4.2.5 Chemotherapy for Local Control 75
4.3 Ewing s Sarcoma 75
4.3.1 Surgery for Ewing s Sarcoma 75
4.3.2 Radiation Therapy for Ewing s Sarcoma! . . 76
4.3.2.1 Radiotherapy Treatment Factors . . 76
4.4 Rhabdomyosarcoma 77
4.4.1 Surgery for Rhabdomyosarcoma . . 77
4.4.2 Radiotherapy for Rhabdomyosarcoma . . . 78
4.4.3 Site Specific Approaches to Local Control . 79
4.4.3.1 Orbit and Head Neck 79
4.4.3.2 Genitourinary: Bladder/Prostate . . 79
4.4.3.3 Vagina and Uterine Sites 80
4.4.3.4 Paratestes 8q
4.4.3.5 Extremity gg
4.4.3.6 Trunk 81
4.5 Non Rhabdomyosarcomatous
Soft Tissue Sarcomas 81
4.5.1 Surgery for Non Rhabdo
Soft Tissue Sarcomas 81
4.5.2 Radiotherapy for Non Rhabdo
Soft Tissue Sarcomas .... 82
4.6 Osteosarcoma 83
4.6.1 Surgery for Osteosarcoma • • ¦ ¦ • ^
4.6.2 Radiotherapy for Osteosarcoma 84
References 84
5 Drug Discovery in Pediatric Bone
and Soft Tissue Sarcomas Using
In Vivo Models
Jennifer K. Peterson, Peter J. Houghton
5.1 Introduction 89
5.2 Pediatric Tumor Models 90
5.3 General Criteria
for Selecting Appropriate Models 91
5.4 Expression Profiles 91
5.5 Criteria for Selecting RMS Xenografts
for Drug Evaluation 92
5.5.1 In Vivo Models in Drug Discovery 93
5.5.2 Models for Optimizing Therapy 94
5.6 Combination Therapy 95
5.7 Molecular Targeted Agents 97
5.8 Combining Signal Transduction Inhibitors . ... 97
5.9 Future Directions 99
References 100
6 Pediatric Rhabdomyosarcoma:
Biology and Results of the North American
Intergroup Rhabdomyosarcoma Trials
Alberto Pappo, Fred G. Barr,
Suzanne L.Wolden
6.1 Epidemiology 104
6.2 Pathology and Biology of Rhabdomyosarcoma . 105
6.2.1 Pathologic Classification
of Rhabdomyosarcoma 105
6.2.2 Chromosomal Translocations
in Alveolar Rhabdomyosarcoma 106
6.2.3 Allelic Loss of 11p15.5
in Embryonal Rhabdomyosarcoma 108
6.2.4 Other Genetic Changes
in Rhabdomyosarcoma 108
6.3 Clinical Presentation and Evaluation
of Extent of Disease 110
6.3.1 Head and Neck Region 110
6.3.2 Genitourinary Tumors 110
6.3.3 Extremity Tumors 110
6.3.4 Trunk 110
6.3.5 Other Sites 111
6.4 Staging 111
6.5 Prognostic Factors 114
6.6 Treatment 114
6.7 Role of Local Therapies 115
6.8 Multi institutional Trials 117
6.8.1 Clinical Group I 117
6.8.2 Clinical Group II 120
6.8.3 Clinical Group III 120
6.8.4 Clinical Group IV 121
6.9 Management of Specific Tumor Sites 121
6.9.1 Parameningeal Tumors 121
6.9.2 OrbitalTumors 121
6.9.3 Head and Neck Non Orbital,
Non Parameningeal Tumors 122
6.9.4 Parotid Tumors 122
6.9.5 Extremity Tumors 123
6.9.6 Paratesticular Rhabdomyosarcoma 123
6.9.7 Female Genital Tract Tumors 123
6.9.8 Bladder and Prostate Rhabdomyosarcoma . 124
6.10 OtherSites 125
6.10.1 Biliary TractTumors 125
6.10.2 Perineal and Perianal Rhabdomyosarcoma . 125
6.10.3 Pelvic and Retroperitoneal
Rhabdomyosarcoma 125
6.11 Acute and Long Term Effects of Therapy 125
6.12 Future Directions 127
6.13 Outcome and Therapy of Relapse 127
References 128
7 Non Rhabdomyosarcoma
Soft Tissue Sarcomas
Sheri L. Spunt, Suzanne L. Wolden,
Deborah E. Schofield, Stephen X. Skapek
7.1 Epidemiology/Pathogenesis 134
7.1.1 Incidence 134
7.1.2 Risk Factors 134
7.2 Pathology/Molecular Pathology 135
7.2.1 Molecular Pathogenesis 137
7.2.2 Synovial Sarcoma
and the SYT/SSX Translation 139
7.2.3 Infantile Fibrosarcoma and ETV6 NTRK3 . . 139
7.2.4 Rhabdoid Tumor and INI1 139
7.2.5 Inflammatory MyofibroblasticTumor
andALK 140
7.2.6 Gastrointestinal Stromal Tumors
andtheKITTransferase 140
7.2.7 Dermatofibrosarcoma Protuberans
andPDGF 140
7.3 Histologic Classification 140
7.3.1 Synovial Sarcoma 141
7.3.2 Malignant Peripheral Nerve Sheath Tumor . 142
7.3.3 Infantile Fibrosarcoma 142
7.3.4 Adult Type Fibrosarcoma 143
7.3.5 Rhabdoid Tumor 143
7.3.6 Inflammatory Myofibroblastic Tumor . ... 144
7.3.7 Gastrointestinal Stromal Tumor 144
7.3.8 Infantile Hemangiopericytoma 145
7.3.9 Desmoplastic Small Round Cell Tumor ... 145
7.3.10 Grading Systems 146
7.4 Clinical Presentation and Diagnosis 146
7.4.1 Presenting Features 146
7.4.2 Differential Diagnosis 146
7.4.3 The Diagnostic Biopsy 147
7.4.4 Evaluation of Disease Extent 147
7.5 Prognostic Factors and Clinical Staging 148
7.5.1 Prognostic Factors 148
7.5.2 Clinical Staging 149
I
7.6 Treatment 150
7.6.1 Surgery 150
7.6.1.1 Surgical Management
of the Primary Tumor 150
7.6.1.2 Surgical Management
ofMetastases 151
7.6.2 Radiation Therapy 151
7.6.2.1 Radiation Planning 152
7.6.2.2 Timing of Radiotherapy 153
7.6.2.3 Brachytherapy 153
7.6.2.4 New Technologies 154
7.6.2.5 Acute Side Effects of Radiotherapy . . 154
7.6.2.6 Long Term Side Effects
of Radiotherapy 154
7.6.3 Chemotherapy 155
7.6.3.1 Adjuvant Chemotherapy 155
7.6.3.2 Neoadjuvant Chemotherapy 156
7.6.3.3 Other Approaches
to the Use of Standard Chemotherapy 157
7.6.3.4 High Dose Chemotherapy 157
7.6.3.5 Novel Agents 157
7.6.3.6 Late Effects of Chemotherapy .... 158
7.7 Current Problems and Future Challenges .... 158
References 158
8 Fibrous and Fibrohistiocytic Tumors
Diane Nam, Benjamin A. Alman
8.1 Fibrous and Fibrohistiocytic Tumors 164
8.2 Soft Tissue Fibrous Tumors 164
8.2.1 Epidemiology/Pathogenesis 165
8.2.2 Pathology/Molecular Biology 166
8.2.3 Clinical Presentation and Diagnosis 166
8.2.4 Prognostic Factors and Clinical Staging . . . 167
8.2.4.1 Treatment of Fibrous Tumors 168
8.2.4.2 Current Problems
and Future Challenges 168
8.3 Fibrohistiocytic Tumors 169
8.4 So Called Fibrohistiocytic Tumors 169
8.4.1 Histologic Classification 169
8.4.2 Benign 170
8.4.2.1 Benign Fibrous Histiocytoma . ... 170
8.4.2.1.1 Epidemiology/Pathogenesis 170
8.4.2.1.2 Pathology/Molecular Pathology ... 171
8.4.2.1.3 Clinical Presentation and Diagnosis . 171
8.4.2.1.4 Prognostic Factors
and Clinical Staging 171
8.4.2.2 Giant Cell Tumor of Tendon Sheath
and Diffuse Type Giant Cell Tumor . 172
8.4.2.2.1 Epidemiology/Pathogenesis 172
8.4.2.2.2 Pathology/Molecular Pathology . . . 172
8.4.2.2.3 Clinical Presentation and Diagnosis . 172
8.4.2.2.4 Prognostic Factors
and Clinical Staging 173
8.4.3 Intermediate 173
8.4.3.1 Giant Cell Fibroblastoma
and Dermatofibrosarcoma
Protuberans 173
8.4.3.1.1 Epidemiology/Pathogenesis 173
8.4.3.1.2 Pathology/Molecular Pathology . . . 173
8.4.3.1.3 Clinical Presentation and Diagnosis . 174
8.4.3.1.4 Prognostic Factors
and Clinical Staging 174
8.4.3.2 Plexiform Fibrohistiocytic Tumor . . 174
8.4.3.2.1 Epidemiology/Pathogenesis 174
8.4.3.2.2 Pathology/Molecular Pathology . . . 174
8.4.3.2.3 Clinical Presentation and Diagnosis . 175
8.4.3.2.4 Prognostic Factors
and Clinical Staging 175
8.4.3.3 Giant Cell Tumor of Soft Tissue ... 175
8.4.3.3.1 Epidemiology/Pathogenesis 175
8.4.3.3.2 Pathology/Molecular Pathology . . . 175
8.4.3.3.3 Clinical Presentation
and Diagnosis 175
8.4.3.3.4 Prognostic Factors
and Clinical Staging 176
8.4.3.3.5 Treatment of Fibrohistiocytic Tumors 176
8.5 Current Problems and Future Challenges .... 176
References 177
9 Ewing Sarcoma Family
ofTumors
Carlos Rodriguez Galindo, Fariba Navid,
Joseph Khoury, Matthew Krasin
9.1 Introduction 181
9.2 Epidemiology 182
9.3 Pathogenesis 182
9.4 Pathology 185
9.4.1 Microscopic Features 185
9.4.2 Molecular Pathology 186
9.5 Clinical Features 188
9.5.1 Extraosseous ESFT 189
9.5.2 Laboratory and Radiologic Evaluation . . 189
9.5.3 Prognostic Factors 190
9.6 Treatment 192
9.6.1 Treatment of Patients
with Localized Disease 192
9.6.1.1 Four Drug Regimens 192
9.6.1.2 Roleof Ifosfamideand Etoposide . 192
9.6.1.3 Increasing Dose Intensity 195
9.6.1.4 Current Studies 199
9.6.2 Local Control in ESFT 199
9.6.2.1 SurgicalTherapy 200
9.6.2.2 Surgery and Adjuvant
Radiation Therapy 201
9.6.2.3 Definitive Radiation Therapy .... 202
9.6.2.4 Role of Systemic Chemotherapy
in Local Control 202
9.6.3 Treatment of Metastatic ESFT 203
9.6.4 MyeloablativeTherapy
with Hematopoietic Stem Cell Rescue
for Metastatic ESFT 205
9.6.5 Second Malignancies 207
9.6.6 Recurrent ESFT 208
9.7 Future Developments 208
References 211
10 Osteosarcoma
Paul A. Meyers
10.1 Incidence 219
10.2 Etiology/Pathogenesis 219
10.3 Molecular Biology 220
10.4 Pathology 221
10.5 Clinical Presentation and Natural History ... 222
10.6 Diagnostic Evaluation 222
10.7 Biopsy 223
10.8 Staging 223
10.9 Prognostic Factors 223
10.10 Treatment 225
10.11 Surgery 227
10.12 Radiation Therapy 227
10.13 Recurrent Disease 228
10.14 Future Directions 228
References 228
Subject Index 235
|
| adam_txt |
1 Epidemiology of Bone
and Soft Tissue Sarcomas
Logan G. Spector, Julie A. Ross,
Rajaram Nagarajan
1.1 Descriptive Epidemiology of Bone Tumors. . 1
1.2 Worldwide Statistics 1
1.3 North American Statistics 3
1.4 Mortality and Survivorship 4
1.5 Etiology of Bone Sarcomas 5
1.6 Bone Sarcomas: Future Directions 7
1.7 Epidemiology of Soft Tissue Sarcomas 7
References 9
2 Pathologic and Molecular Techniques
Used in the Diagnosis
and Treatment Planning of Sarcomas
Timothy Triche, Deborah Schofield
2.1 Introduction 13
2.2 Standard Histopathology 14
2.2.1 Light Microscopy 14
2.2.2 Immunohistochemistry 15
2.2.3 Electron Microscopy 16
2.3 Cytogenetics 16
2.3.1 Classical Cytogenetic Analysis 16
2.3.2 In Situ Hybridization 18
2.3.3 Spectral Karyotyping 19
2.3.4 Comparative Genomic Hybridization . 20
2.4 Molecular Genetic Techniques 20
2.4.1 Introduction 20
2.4.2 PCRandQ PCR 20
2.4.3 Microarrays 23
2.4.4 Proteomics 27
2.4.5 Conclusion 30
References 31
I
3 Diagnostic Imaging of Pediatric Bone
and Soft Tissue Sarcomas
Thomas D. Henry, Mary E. McCarville,
Fredric A. Hoffer
3.1 Introduction 35
3.2 Initial Evaluation of Bone
and Soft Tissue Sarcomas 35
3.3 Imaging Techniques
for Evaluating the Primary Tumor 37
3.4 Staging 38
3.4.1 Local Extent of the Tumor 38
3.4.2 Detection of Metastatic Disease 40
3.5 Criteria for Evaluating Treatment Response . 41
3.6 Outcome Criteria 43
3.7 Biopsy of the Primary Lesion 43
3.8 Imaging Characteristics of Specific Tumors . 44
3.8.1 Bone Sarcomas 44
3.8.1.1 Osteosarcoma 44
3.8.1.2 Chondrosarcoma 48
3.8.1.3 Ewing Sarcoma of Bone 48
3.8.2 Soft Tissue Sarcomas 51
3.8.2.1 Differentiating Benign
from Malignant Soft Tissue Lesions 51
3.8.2.2 Soft Tissue Ewing Sarcoma Family
ofTumors 52
3.8.2.3 Rhabdomyosarcoma 54
3.8.2.4 Non rhabdomyosarcoma
Soft Tissue Sarcomas 61
3.8.2.4.1 Synovial Sarcoma 61
3.8.2.4.2 Congenital Fibrosarcoma 62
3.8.2.4.3 Hemangiopericytoma 63
3.8.2.4.4 Malignant Peripheral Nerve
Sheath Tumors IMPNST) 65
3.9 Post treatment Imaging Concerns 66
3.10 Radiofrequency Ablation 66
References 67
m
4 Local Control Issues in Pediatric Bone
and Soft Tissue Sarcomas
John C. Breneman, David Rodeberg,
Ruth F. Lavigne, Ken Brown, Eugene S. Wiener
4.1 Significance of Local Control
in Pediatric Sarcomas 71
4.2 Background to Local Control Modalities 72
4.2.1 Patterns of Tumor Growth 72
4.2.2 Surgery 72
4.2.2.1 Biopsy Guidelines 72
4.2.2.2 Surgical Margins 72
4.2.3 Radiation Therapy . 73
4.2.3.1 Types of Radiation 73
4.2.3.2 Techniques of Radiotherapy . 73
4.2.3.3 Treatment Morbidity 74
4.2.4 Combined Surgery and Radiotherapy
for Local Control 74
4.2.5 Chemotherapy for Local Control 75
4.3 Ewing's Sarcoma 75
4.3.1 Surgery for Ewing's Sarcoma 75
4.3.2 Radiation Therapy for Ewing's Sarcoma! '. '. 76
4.3.2.1 Radiotherapy Treatment Factors . . 76
4.4 Rhabdomyosarcoma 77
4.4.1 Surgery for Rhabdomyosarcoma . . 77
4.4.2 Radiotherapy for Rhabdomyosarcoma . '. . 78
4.4.3 Site Specific Approaches to Local Control . 79
4.4.3.1 Orbit and Head Neck 79
4.4.3.2 Genitourinary: Bladder/Prostate . . 79
4.4.3.3 Vagina and Uterine Sites 80
4.4.3.4 Paratestes 8q
4.4.3.5 Extremity gg
4.4.3.6 Trunk 81
4.5 Non Rhabdomyosarcomatous
Soft Tissue Sarcomas 81
4.5.1 Surgery for Non Rhabdo
Soft Tissue Sarcomas 81
4.5.2 Radiotherapy for Non Rhabdo
Soft Tissue Sarcomas . 82
4.6 Osteosarcoma 83
4.6.1 Surgery for Osteosarcoma • • ¦ ¦ • ^
4.6.2 Radiotherapy for Osteosarcoma 84
References 84
5 Drug Discovery in Pediatric Bone
and Soft Tissue Sarcomas Using
In Vivo Models
Jennifer K. Peterson, Peter J. Houghton
5.1 Introduction 89
5.2 Pediatric Tumor Models 90
5.3 General Criteria
for Selecting Appropriate Models 91
5.4 Expression Profiles 91
5.5 Criteria for Selecting RMS Xenografts
for Drug Evaluation 92
5.5.1 In Vivo Models in Drug Discovery 93
5.5.2 Models for Optimizing Therapy 94
5.6 Combination Therapy 95
5.7 Molecular Targeted Agents 97
5.8 Combining Signal Transduction Inhibitors . . 97
5.9 Future Directions 99
References 100
6 Pediatric Rhabdomyosarcoma:
Biology and Results of the North American
Intergroup Rhabdomyosarcoma Trials
Alberto Pappo, Fred G. Barr,
Suzanne L.Wolden
6.1 Epidemiology 104
6.2 Pathology and Biology of Rhabdomyosarcoma . 105
6.2.1 Pathologic Classification
of Rhabdomyosarcoma 105
6.2.2 Chromosomal Translocations
in Alveolar Rhabdomyosarcoma 106
6.2.3 Allelic Loss of 11p15.5
in Embryonal Rhabdomyosarcoma 108
6.2.4 Other Genetic Changes
in Rhabdomyosarcoma 108
6.3 Clinical Presentation and Evaluation
of Extent of Disease 110
6.3.1 Head and Neck Region 110
6.3.2 Genitourinary Tumors 110
6.3.3 Extremity Tumors 110
6.3.4 Trunk 110
6.3.5 Other Sites 111
6.4 Staging 111
6.5 Prognostic Factors 114
6.6 Treatment 114
6.7 Role of Local Therapies 115
6.8 Multi institutional Trials 117
6.8.1 Clinical Group I 117
6.8.2 Clinical Group II 120
6.8.3 Clinical Group III 120
6.8.4 Clinical Group IV 121
6.9 Management of Specific Tumor Sites 121
6.9.1 Parameningeal Tumors 121
6.9.2 OrbitalTumors 121
6.9.3 Head and Neck Non Orbital,
Non Parameningeal Tumors 122
6.9.4 Parotid Tumors 122
6.9.5 Extremity Tumors 123
6.9.6 Paratesticular Rhabdomyosarcoma 123
6.9.7 Female Genital Tract Tumors 123
6.9.8 Bladder and Prostate Rhabdomyosarcoma . 124
6.10 OtherSites 125
6.10.1 Biliary TractTumors 125
6.10.2 Perineal and Perianal Rhabdomyosarcoma . 125
6.10.3 Pelvic and Retroperitoneal
Rhabdomyosarcoma 125
6.11 Acute and Long Term Effects of Therapy 125
6.12 Future Directions 127
6.13 Outcome and Therapy of Relapse 127
References 128
7 Non Rhabdomyosarcoma
Soft Tissue Sarcomas
Sheri L. Spunt, Suzanne L. Wolden,
Deborah E. Schofield, Stephen X. Skapek
7.1 Epidemiology/Pathogenesis 134
7.1.1 Incidence 134
7.1.2 Risk Factors 134
7.2 Pathology/Molecular Pathology 135
7.2.1 Molecular Pathogenesis 137
7.2.2 Synovial Sarcoma
and the SYT/SSX Translation 139
7.2.3 Infantile Fibrosarcoma and ETV6 NTRK3 . . 139
7.2.4 Rhabdoid Tumor and INI1 139
7.2.5 Inflammatory MyofibroblasticTumor
andALK 140
7.2.6 Gastrointestinal Stromal Tumors
andtheKITTransferase 140
7.2.7 Dermatofibrosarcoma Protuberans
andPDGF 140
7.3 Histologic Classification 140
7.3.1 Synovial Sarcoma 141
7.3.2 Malignant Peripheral Nerve Sheath Tumor . 142
7.3.3 Infantile Fibrosarcoma 142
7.3.4 Adult Type Fibrosarcoma 143
7.3.5 Rhabdoid Tumor 143
7.3.6 Inflammatory Myofibroblastic Tumor . . 144
7.3.7 Gastrointestinal Stromal Tumor 144
7.3.8 Infantile Hemangiopericytoma 145
7.3.9 Desmoplastic Small Round Cell Tumor . 145
7.3.10 Grading Systems 146
7.4 Clinical Presentation and Diagnosis 146
7.4.1 Presenting Features 146
7.4.2 Differential Diagnosis 146
7.4.3 The Diagnostic Biopsy 147
7.4.4 Evaluation of Disease Extent 147
7.5 Prognostic Factors and Clinical Staging 148
7.5.1 Prognostic Factors 148
7.5.2 Clinical Staging 149
I
7.6 Treatment 150
7.6.1 Surgery 150
7.6.1.1 Surgical Management
of the Primary Tumor 150
7.6.1.2 Surgical Management
ofMetastases 151
7.6.2 Radiation Therapy 151
7.6.2.1 Radiation Planning 152
7.6.2.2 Timing of Radiotherapy 153
7.6.2.3 Brachytherapy 153
7.6.2.4 New Technologies 154
7.6.2.5 Acute Side Effects of Radiotherapy . . 154
7.6.2.6 Long Term Side Effects
of Radiotherapy 154
7.6.3 Chemotherapy 155
7.6.3.1 Adjuvant Chemotherapy 155
7.6.3.2 Neoadjuvant Chemotherapy 156
7.6.3.3 Other Approaches
to the Use of Standard Chemotherapy 157
7.6.3.4 High Dose Chemotherapy 157
7.6.3.5 Novel Agents 157
7.6.3.6 Late Effects of Chemotherapy . 158
7.7 Current Problems and Future Challenges . 158
References 158
8 Fibrous and Fibrohistiocytic Tumors
Diane Nam, Benjamin A. Alman
8.1 Fibrous and Fibrohistiocytic Tumors 164
8.2 Soft Tissue Fibrous Tumors 164
8.2.1 Epidemiology/Pathogenesis 165
8.2.2 Pathology/Molecular Biology 166
8.2.3 Clinical Presentation and Diagnosis 166
8.2.4 Prognostic Factors and Clinical Staging . . . 167
8.2.4.1 Treatment of Fibrous Tumors 168
8.2.4.2 Current Problems
and Future Challenges 168
8.3 Fibrohistiocytic Tumors 169
8.4 So Called Fibrohistiocytic Tumors 169
8.4.1 Histologic Classification 169
8.4.2 Benign 170
8.4.2.1 Benign Fibrous Histiocytoma . . 170
8.4.2.1.1 Epidemiology/Pathogenesis 170
8.4.2.1.2 Pathology/Molecular Pathology . 171
8.4.2.1.3 Clinical Presentation and Diagnosis . 171
8.4.2.1.4 Prognostic Factors
and Clinical Staging 171
8.4.2.2 Giant Cell Tumor of Tendon Sheath
and Diffuse Type Giant Cell Tumor . 172
8.4.2.2.1 Epidemiology/Pathogenesis 172
8.4.2.2.2 Pathology/Molecular Pathology . . . 172
8.4.2.2.3 Clinical Presentation and Diagnosis . 172
8.4.2.2.4 Prognostic Factors
and Clinical Staging 173
8.4.3 Intermediate 173
8.4.3.1 Giant Cell Fibroblastoma
and Dermatofibrosarcoma
Protuberans 173
8.4.3.1.1 Epidemiology/Pathogenesis 173
8.4.3.1.2 Pathology/Molecular Pathology . . . 173
8.4.3.1.3 Clinical Presentation and Diagnosis . 174
8.4.3.1.4 Prognostic Factors
and Clinical Staging 174
8.4.3.2 Plexiform Fibrohistiocytic Tumor . . 174
8.4.3.2.1 Epidemiology/Pathogenesis 174
8.4.3.2.2 Pathology/Molecular Pathology . . . 174
8.4.3.2.3 Clinical Presentation and Diagnosis . 175
8.4.3.2.4 Prognostic Factors
and Clinical Staging 175
8.4.3.3 Giant Cell Tumor of Soft Tissue . 175
8.4.3.3.1 Epidemiology/Pathogenesis 175
8.4.3.3.2 Pathology/Molecular Pathology . . . 175
8.4.3.3.3 Clinical Presentation
and Diagnosis 175
8.4.3.3.4 Prognostic Factors
and Clinical Staging 176
8.4.3.3.5 Treatment of Fibrohistiocytic Tumors 176
8.5 Current Problems and Future Challenges . 176
References 177
9 Ewing Sarcoma Family
ofTumors
Carlos Rodriguez Galindo, Fariba Navid,
Joseph Khoury, Matthew Krasin
9.1 Introduction 181
9.2 Epidemiology 182
9.3 Pathogenesis 182
9.4 Pathology 185
9.4.1 Microscopic Features 185
9.4.2 Molecular Pathology 186
9.5 Clinical Features 188
9.5.1 Extraosseous ESFT 189
9.5.2 Laboratory and Radiologic Evaluation . . 189
9.5.3 Prognostic Factors 190
9.6 Treatment 192
9.6.1 Treatment of Patients
with Localized Disease 192
9.6.1.1 Four Drug Regimens 192
9.6.1.2 Roleof Ifosfamideand Etoposide . 192
9.6.1.3 Increasing Dose Intensity 195
9.6.1.4 Current Studies 199
9.6.2 Local Control in ESFT 199
9.6.2.1 SurgicalTherapy 200
9.6.2.2 Surgery and Adjuvant
Radiation Therapy 201
9.6.2.3 Definitive Radiation Therapy . 202
9.6.2.4 Role of Systemic Chemotherapy
in Local Control 202
9.6.3 Treatment of Metastatic ESFT 203
9.6.4 MyeloablativeTherapy
with Hematopoietic Stem Cell Rescue
for Metastatic ESFT 205
9.6.5 Second Malignancies 207
9.6.6 Recurrent ESFT 208
9.7 Future Developments 208
References 211
10 Osteosarcoma
Paul A. Meyers
10.1 Incidence 219
10.2 Etiology/Pathogenesis 219
10.3 Molecular Biology 220
10.4 Pathology 221
10.5 Clinical Presentation and Natural History . 222
10.6 Diagnostic Evaluation 222
10.7 Biopsy 223
10.8 Staging 223
10.9 Prognostic Factors 223
10.10 Treatment 225
10.11 Surgery 227
10.12 Radiation Therapy 227
10.13 Recurrent Disease 228
10.14 Future Directions 228
References 228
Subject Index 235 |
| any_adam_object | 1 |
| any_adam_object_boolean | 1 |
| author2 | Pappo, Alberto |
| author2_role | edt |
| author2_variant | a p ap |
| author_facet | Pappo, Alberto |
| building | Verbundindex |
| bvnumber | BV022378190 |
| classification_rvk | XH 9224 |
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| ctrlnum | (OCoLC)315473637 (DE-599)BVBBV022378190 |
| discipline | Medizin |
| discipline_str_mv | Medizin |
| doi_str_mv | 10.1007/3-540-29447-3 |
| format | Electronic eBook |
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| illustrated | Not Illustrated |
| index_date | 2024-07-02T17:10:12Z |
| indexdate | 2024-08-01T10:50:08Z |
| institution | BVB |
| isbn | 9783540294474 |
| language | German |
| oai_aleph_id | oai:aleph.bib-bvb.de:BVB01-015587218 |
| oclc_num | 315473637 |
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| publishDate | 2006 |
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| publisher | Springer |
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| series2 | Pediatric oncology |
| spellingShingle | Pediatric bone and soft tissue sarcomas Kind (DE-588)4030550-8 gnd Knochentumor (DE-588)4123535-6 gnd Weichteilsarkom (DE-588)4189415-7 gnd |
| subject_GND | (DE-588)4030550-8 (DE-588)4123535-6 (DE-588)4189415-7 (DE-588)4143413-4 |
| title | Pediatric bone and soft tissue sarcomas |
| title_auth | Pediatric bone and soft tissue sarcomas |
| title_exact_search | Pediatric bone and soft tissue sarcomas |
| title_exact_search_txtP | Pediatric bone and soft tissue sarcomas |
| title_full | Pediatric bone and soft tissue sarcomas Alberto Pappo (ed.) |
| title_fullStr | Pediatric bone and soft tissue sarcomas Alberto Pappo (ed.) |
| title_full_unstemmed | Pediatric bone and soft tissue sarcomas Alberto Pappo (ed.) |
| title_short | Pediatric bone and soft tissue sarcomas |
| title_sort | pediatric bone and soft tissue sarcomas |
| topic | Kind (DE-588)4030550-8 gnd Knochentumor (DE-588)4123535-6 gnd Weichteilsarkom (DE-588)4189415-7 gnd |
| topic_facet | Kind Knochentumor Weichteilsarkom Aufsatzsammlung |
| url | https://doi.org/10.1007/3-540-29447-3 http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=015587218&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
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