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Management of Soft Tissue Sarcoma
Brennan, Antonescu, Maki
Editore
Springer
Anno
2013
Pagine
380
ISBN
9781461450030
150,00 €
I prezzi indicati possono subire variazioni poiché soggetti all'oscillazione dei cambi delle valute e/o agli aggiornamenti effettuati dagli Editori.
  • Written by world renowned experts
    •  
  • Explains histopathology as a determinant of current treatment modalities
    •  
  • Covers surgical treatment, chemotherapy, radiation therapy and novel pharmacotherapy
    •  
  • Gives a practical up to date approach to all the different subtypes of adult soft tissue sarcoma

Management of Soft Tissue Sarcoma addresses the diagnosis and best current management of adult soft tissue sarcomas. Edited by world renowned experts, this book delineates and discusses each different sarcoma subtype individually. Both clinical and molecular diagnoses are addressed, and tumor histopathology is employed as the basis of treatment recommendations including surgery, radiation therapy, systemic therapy and novel therapeutics.

 

ix
Contents
1 General Description ............................................................................... 1
Introduction ................................................................................................ 1
Incidence and Prevalence ........................................................................... 3
Predisposing and Genetic Factors .............................................................. 8
References .................................................................................................. 13
2 Natural History: Importance of Size, Site,
and Histopathology .................................................................................. 19
Natural History ........................................................................................... 19
Influence of Site ......................................................................................... 19
Staging ....................................................................................................... 19
Staging of Retroperitoneal Visceral Sarcoma ............................................ 26
Prognostic Factors for Extremity and Superficial
Soft Tissue Sarcoma ................................................................................... 30
Disease-Specific Survival .......................................................................... 31
Prognostic Factors for Survival Following Local
Recurrence of Extremity Sarcoma ............................................................. 32
Prognostic Factors: Nomograms ................................................................ 32
References .................................................................................................. 34
3 General Statement as to Efficacy of Surgery/
Chemotherapy/Radiation Therapy ........................................................ 37
Extent of Primary Surgery ......................................................................... 37
Surgical Treatment of Local Recurrence ................................................... 39
Diagnostic Imaging .................................................................................... 39
Surgery for Metastatic Disease .................................................................. 41
Pulmonary Metastasis ............................................................................ 41
Radiation Therapy ...................................................................................... 49
Adjuvant Radiation Therapy .................................................................. 49
Dose of Radiation Therapy .................................................................... 51
Adjuvant Brachytherapy ........................................................................ 51
x Contents
Radiation Therapy in the Presence of Positive
Microscopic Margins ............................................................................. 52
Definitive Radiation Therapy ................................................................. 52
Radiation to Patients with Neurovascular Involvement ......................... 52
Wound Complications of Radiation Therapy ......................................... 53
Sarcomas More Common in Adults ....................................................... 54
Larger Randomized Studies ................................................................... 54
Selected Meta-analyses of Randomized Trials
of Adjuvant Chemotherapy .................................................................... 56
Adjuvant Therapy for GIST ................................................................... 57
Sarcomas More Common in the Pediatric Setting ................................. 58
Special Techniques for Primary and Locally Recurrent Disease ............... 60
Intra-arterial Chemotherapy ................................................................... 60
Limb Perfusion and Hyperthermia ......................................................... 60
References .................................................................................................. 61
Part I Management by Histopathology: Introduction
4 Gastrointestinal Stromal Tumors (GISTs) ............................................. 67
Imaging ...................................................................................................... 67
Familial GIST ............................................................................................ 67
Natural History ........................................................................................... 72
Diagnosis, Molecular Pathology ................................................................ 73
Treatment ................................................................................................... 74
Adjuvant Imatinib for Primary GIST ..................................................... 74
Neoadjuvant Therapy for Primary Disease Not
Amenable to Surgery ............................................................................. 77
Treatment of Recurrence ........................................................................ 77
Systemic Targeted Therapy for Metastatic GIST ................................... 77
Dose Intensity over Time ....................................................................... 82
Imatinib Pharmacokinetics ..................................................................... 83
Second-Line Sunitinib for Imatinib-Resistant Metastatic GIST ................ 84
Other Tyrosine Kinase Inhibitors for Metastatic GIST
Failing Imatinib and Sunitinib ............................................................... 85
Newer Agents ............................................................................................. 85
References .................................................................................................. 87
5 Liposarcoma ............................................................................................. 93
Imaging ...................................................................................................... 97
Diagnosis .................................................................................................... 99
Treatment ................................................................................................... 99
Radiation Therapy ...................................................................................... 103
Systemic Therapy: General Considerations ............................................... 104
Adjuvant Therapy ...................................................................................... 105
Treatment of Metastatic Disease ................................................................ 105
Contents xi
Outcomes ................................................................................................... 107
Outcome Following Metastasis .................................................................. 109
References .................................................................................................. 110
6 Leiomyosarcoma .................................................................................... 113
Imaging ...................................................................................................... 113
Diagnosis, Molecular Pathology ................................................................ 115
Treatment ................................................................................................... 117
Radiation Therapy ...................................................................................... 120
Systemic Therapy ....................................................................................... 120
Adjuvant Chemotherapy ........................................................................ 120
Treatment of Recurrence ............................................................................ 121
Metastatic Disease ..................................................................................... 121
Outcome ..................................................................................................... 123
References .................................................................................................. 124
7 Undifferentiated Pleomorphic Sarcoma
(UPS; Malignant Fibrous Histiocytoma: MFH)
and Myxofi brosarcoma ............................................................................ 129
Imaging ...................................................................................................... 129
Diagnosis/Molecular Pathology ................................................................. 129
Natural History ........................................................................................... 131
Treatment ................................................................................................... 132
Radiation Therapy ...................................................................................... 132
Metastatic Disease ..................................................................................... 132
Adjuvant Chemotherapy ............................................................................ 133
Outcome ..................................................................................................... 134
References .................................................................................................. 135
8 Synovial Sarcoma ..................................................................................... 137
Imaging ...................................................................................................... 137
Diagnosis, Molecular Pathology ................................................................ 137
Treatment ................................................................................................... 141
Radiation Therapy ...................................................................................... 141
Chemotherapy ............................................................................................ 141
Treatment of Recurrence ............................................................................ 143
Local Recurrence ................................................................................... 143
Systemic Treatment ................................................................................ 143
Outcome ..................................................................................................... 145
References .................................................................................................. 146
9 Malignant Peripheral Nerve Sheath Tumor
(MPNST) and Triton Tumor ................................................................... 149
Presentation ................................................................................................ 149
Imaging ...................................................................................................... 149
Diagnosis/Pathology .................................................................................. 152
Neurofibromatosis Type 1 and Outcome ................................................... 154
xii Contents
Treatment ................................................................................................... 154
Radiation Therapy ...................................................................................... 154
Chemotherapy ............................................................................................ 155
Outcome ..................................................................................................... 157
References .................................................................................................. 158
10 Desmoid Tumor/Deep-Seated Fibromatosis
(Desmoid-Type Fibromatosis) ................................................................. 161
Clinical Presentation .................................................................................. 163
Imaging ...................................................................................................... 163
Diagnosis: Molecular ................................................................................. 163
Natural History ........................................................................................... 165
Treatment ................................................................................................... 166
Treatment of Recurrence ............................................................................ 166
Systemic Therapy ....................................................................................... 167
Treatment by Observation .......................................................................... 169
Radiation Therapy ...................................................................................... 169
Patterns of Failure ...................................................................................... 170
Outcome ..................................................................................................... 171
References .................................................................................................. 175
11 Solitary Fibrous Tumor/Hemangiopericytoma ..................................... 179
Outcome ..................................................................................................... 182
References .................................................................................................. 183
12 Fibrosarcoma and Its Variants ............................................................... 185
Outcome ..................................................................................................... 185
Dermatofibrosarcoma Protuberans ............................................................. 185
Outcome ..................................................................................................... 188
Low-Grade Fibromyxoid Sarcoma (Evans’ Tumor) .................................. 189
Outcome ..................................................................................................... 194
Sclerosing Epithelioid Fibrosarcoma ......................................................... 194
Inflammatory Myofibroblastic Tumor........................................................ 195
Infantile Fibrosarcoma ............................................................................... 196
Myxoinflammatory Fibroblastic Sarcoma/Inflammatory
Myxohyaline Tumor of Distal Extremities ................................................ 197
Adult-Type Fibrosarcoma .......................................................................... 197
References .................................................................................................. 198
13 Vascular Sarcomas ................................................................................... 201
Epithelioid Hemangioendothelioma .......................................................... 201
Angiosarcoma/Lymphangiosarcoma .......................................................... 204
Outcome ..................................................................................................... 209
Kaposi Sarcoma ......................................................................................... 210
References .................................................................................................. 211
Contents xiii
14 Epithelioid Sarcoma ................................................................................. 215
Outcome ..................................................................................................... 217
References .................................................................................................. 219
15 Sarcomas More Common in Children ................................................... 221
Soft Tissue Sarcomas More Commonly Observed
in Pediatric Patients .................................................................................... 221
Ewing Sarcoma Family of Tumors (EFT) ................................................. 222
Demographics ............................................................................................ 226
Primary Therapy ........................................................................................ 226
Adjuvant Chemotherapy ............................................................................ 228
High-Dose Systemic Therapy for Metastatic Disease ............................... 229
Standard Cytotoxic Chemotherapy After Disease Relapse ........................ 230
Investigational Approaches ........................................................................ 231
Ewing Sarcoma-Like Small Round Cell Tumors....................................... 232
Rhabdomyosarcoma ................................................................................... 233
Demographics ............................................................................................ 234
Molecular Biology ..................................................................................... 236
Risk Stratification....................................................................................... 238
Staging ....................................................................................................... 238
Imaging ...................................................................................................... 240
Primary Therapy ........................................................................................ 240
Chemotherapy for Metastatic Disease ....................................................... 242
Embryonal Sarcoma ................................................................................... 243
Calcifying Nested Stromal and Epithelial Tumor ...................................... 244
References .................................................................................................. 245
16 Radiation-Induced Sarcomas .................................................................. 251
References .................................................................................................. 256
17 Alveolar Soft Part Sarcoma .................................................................... 259
Imaging ...................................................................................................... 259
Diagnosis/Molecular Pathology ................................................................. 259
Primary Treatment ..................................................................................... 262
Treatment of Metastatic Disease ................................................................ 262
Outcome ..................................................................................................... 263
References .................................................................................................. 264
18 Clear Cell Sarcoma/Melanoma of Soft Parts ........................................ 267
Imaging ...................................................................................................... 267
Diagnosis: Molecular Pathology ................................................................ 267
Treatment ................................................................................................... 270
Outcome ..................................................................................................... 271
References .................................................................................................. 272
xiv Contents
19 Desmoplastic Small Round Cell Tumor ................................................. 275
Imaging ...................................................................................................... 275
Diagnosis .................................................................................................... 275
Treatment ................................................................................................... 276
Outcome ..................................................................................................... 278
References .................................................................................................. 279
20 Extraskeletal Myxoid Chondrosarcoma ................................................ 281
Imaging ...................................................................................................... 281
Diagnosis .................................................................................................... 281
Treatment ................................................................................................... 285
Outcome ..................................................................................................... 285
References .................................................................................................. 286
21 Other Uterine Sarcomas .......................................................................... 289
Endometrial Stromal Sarcoma ................................................................... 289
Diagnosis ................................................................................................ 289
Treatment ............................................................................................... 290
Outcome ................................................................................................. 291
Undifferentiated Endometrial Sarcoma ..................................................... 293
Outcome ................................................................................................. 294
Uterine Carcinosarcomas and Other Malignant
Mixed Müllerian Tumors ........................................................................... 294
PEComas .................................................................................................... 296
Outcome ................................................................................................. 296
References .................................................................................................. 297
22 Extraskeletal Osteogenic Sarcoma ......................................................... 301
Imaging ...................................................................................................... 301
Diagnosis .................................................................................................... 301
Treatment ................................................................................................... 305
Outcome ..................................................................................................... 305
References .................................................................................................. 307
23 Sustentacular Tumors of Lymph Tissue ................................................. 309
Follicular Dendritic Cell Tumor (Dendritic Reticulum
Cell Tumor [FDCT]) and Interdigitating Reticulum
Cell Tumor (IDRCT).................................................................................. 312
True Histiocytic Sarcoma ........................................................................... 312
Langerhans Cell Tumors ............................................................................ 313
Outcome ..................................................................................................... 313
References .................................................................................................. 315
24 Uncommon/Unique Sites ......................................................................... 317
Heart and Great Vessels ............................................................................. 317
Primary Sarcomas of the Breast ................................................................. 318
Phyllodes Tumor .................................................................................... 321
Diagnosis ................................................................................................ 322
Outcome ................................................................................................. 323
Contents xv
Head and Neck ........................................................................................... 323
Treatment ............................................................................................... 324
Primary Sarcomas of the Mediastinum ...................................................... 324
Liver ........................................................................................................... 325
References .................................................................................................. 325
Part II Benign and Less Aggressive Lesions: Introduction
25 Mostly Benign/Rarely Metastasizing Soft Tissue Tumor ..................... 329
Ossifying Fibromyxoid Tumor .................................................................. 329
Perivascular Epithelioid Cell Tumor (PEComa)
and Related Entities: Lymphangioleiomyomatosis,
Angiomyolipoma, and Sugar Cell Tumor .................................................. 330
Therapy ...................................................................................................... 331
Giant Cell Tumor of Tendon Sheath/Pigmented
Villonodular Synovitis ............................................................................... 332
Myoepithelioma of Soft Tissue .................................................................. 334
Glomus Tumor ........................................................................................... 334
References .................................................................................................. 336
26 Benign Soft Tissue Tumors ...................................................................... 339
Lipoma ....................................................................................................... 339
Lipomatosis ................................................................................................ 340
Lipoblastoma/Lipoblastomatosis ............................................................... 340
Angiolipoma .............................................................................................. 341
Angiomyolipoma ....................................................................................... 341
Angiomyelolipoma .................................................................................... 343
Hibernoma .................................................................................................. 343
Elastofibroma ............................................................................................. 345
Granular Cell Tumor .................................................................................. 347
Hemangioma .............................................................................................. 347
Leiomyoma ................................................................................................ 350
Schwannoma .............................................................................................. 350
Neurofibroma ............................................................................................. 350
Myxoma ..................................................................................................... 351
Angiomyxoma ............................................................................................ 352
References .................................................................................................. 353
27 Reactive Lesions ....................................................................................... 355
Myositis Ossificans .................................................................................... 355
Nodular Fasciitis ........................................................................................ 355
Sarcoma Masquerade ................................................................................. 356
References .................................................................................................. 358
Suggested Keywords ........................................................................................ 359
Index .................................................................................................................. 369
 

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