TUMORS OF THE BONES AND JOINTS
1. Introduction...... 1 Structure of Normal Bone ...... 1 Development of Bone ...... 2 Intramembranous Ossification ...... 2 Endochondral Ossification...... 3 Classification of Bone Tumors...... 3 Incidence of Bone Tumors...... 3 Methods of Biopsy...... 4 Open Biopsy...... 4 Needle Biopsy...... 5 Fine-Needle Aspiration Biopsy...... 5 Handling of Specimens ...... 6 Decalcification...... 7 Assessment of Chemotherapy Effect...... 7 Grading of Bone Tumors ...... 8 Staging of Bone Tumors...... 8 2. Genetics of Bone Tumors...... 11 Introduction...... 11 Basic Principles of Cancer Cytogenetics ...... 11 Cytogenetic Analysis ...... 11 Specimen Requirements...... 11 Cell Culture and Chromosome Banding...... 11 Nomenclature...... 12 Molecular Cytogenetics...... 13 Specimen ...... 13 Probes...... 15 Technical Variations...... 15 Molecular Biology...... 16 Oncogenes...... 16 Oncogene Detection ...... 17 Gene Amplification...... 17 Tumor Suppressor Genes...... 20 DNA Mismatch Repair Gene Defects...... 21 Microarray Technology ...... 22 3. Radiographic Appearance of Bone Tumors ...... 27 Location...... 27
ix Tumors of the Bones and Joints
Size ...... 28 Internal Margins...... 28 Matrix Pattern ...... 29 Proliferative Bone Induction...... 30 Interrupted Periosteal Reaction...... 32 Radionuclide Bone Imaging...... 32 Computerized Tomograms...... 33 Magnetic Resonance Imaging ...... 34 4. Cartilaginous Lesions...... 37 Osteochondroma...... 37 Multiple Hereditary Exostoses...... 46 Enchondroma...... 46 Multiple Chondromas...... 52 Periosteal Chondroma...... 56 Soft Tissue Chondroma ...... 59 Chondroblastoma...... 61 Chondromyxoid Fibroma ...... 67 Chondrosarcoma...... 73 Chondrosarcoma of the Small Bones of the Hands and Feet...... 86 Periosteal Chondrosarcoma...... 87 Secondary Chondrosarcoma ...... 89 Dedifferentiated Chondrosarcoma ...... 91 Mesenchymal Chondrosarcoma ...... 99 Clear Cell Chondrosarcoma...... 104 5. Bone-Forming Lesions ...... 119 Benign Bone-Forming Tumors...... 119 Osteoid Osteoma...... 119 Osteoblastoma...... 126 Malignant Bone-Forming Tumors...... 135 Osteosarcoma of Bone...... 135 Medullary Osteosarcomas ...... 136 Conventional Osteosarcoma...... 136 Osteosarcoma of Jawbones...... 149 Osteosarcoma in Paget’s Disease ...... 150 Postradiation Sarcoma...... 151 Osteosarcoma in Other Benign Condtions...... 152 Multicentric Osteosarcoma ...... 154 Telangiectatic Osteosarcoma...... 154
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Small Cell Osteosarcoma...... 158 Low-Grade Osteosarcoma ...... 160 Genetics and Other Special Techniques...... 162 Genetic Predisposition to Osteosarcoma...... 162 Prognostic Markers...... 165 Other Potential Genetic Lesions ...... 168 Tumor Cytogenetics...... 168 Treatment and Prognosis for Patients with Osteosarcomas...... 169 Osteosarcomas of the Surface of Bone...... 171 Parosteal Osteosarcoma...... 171 Periosteal Osteosarcoma...... 176 High-Grade Surface Osteosarcoma...... 180 6. Fibrogenic Tumors ...... 193 Desmoplastic Fibroma...... 193 Fibrosarcoma ...... 196 7. “Histiocytic” Tumors ...... 201 Benign Fibrous Histiocytoma...... 201 Malignant Fibrous Histiocytoma...... 202 8. Small Cell Malignancies...... 209 Ewing’s Sarcoma...... 209 Myeloma...... 222 Multiple Myeloma...... 222 Solitary Myeloma (Plasmacytoma) ...... 229 Osteosclerotic Myeloma...... 230 Malignant Lymphoma...... 232 9. Notochordal Tumors ...... 249 Chordoma ...... 249 Chondroid Chordoma...... 256 Dedifferentiated Chordoma...... 257 10. Vascular Tumors...... 261 Benign Vascular Tumors...... 261 Hemangioma...... 261 Massive Osteolysis...... 264 Glomus Tumor...... 265 Malignant Vascular Tumors...... 266 Angiosarcoma...... 266 Epithelioid Hemangioendothelioma...... 273 Hemangiopericytoma...... 276
xi Tumors of the Bones and Joints
11. Giant Cell Tumor...... 281 Giant Cell Tumor...... 281 Malignancy in Giant Cell Tumors...... 292 12. Adamantinoma of Long Bones...... 299 Adamantinoma...... 299 13. Miscellaneous Tumors ...... 309 Neurilemmoma of Bone...... 309 Lipoma of Bone ...... 311 Liposarcoma...... 313 Clear Cell Sarcoma...... 314 Alveolar Soft Part Sarcoma...... 315 Leiomyosarcoma...... 316 14. Conditions that Simulate Primary Neoplasms of Bone...... 321 Metastatic Malignancy...... 321 Cystic Lesions of Bone that Simulate Neoplasms ...... 324 Aneurysmal Bone Cyst...... 324 Simple Cyst...... 330 Intraosseous Ganglion...... 330 Epidermoid Cyst...... 332 Subungual Keratoacanthoma...... 333 Fibrous Lesions of Bone that Simulate Neoplasms ...... 334 Metaphyseal Fibrous Defect...... 334 Periosteal Desmoid (Avulsive Cortical Irregularity)...... 336 Fibrous Dysplasia...... 337 Osteofibrous Dysplasia...... 343 Congenital Fibromatosis (Myofibromatosis)...... 345 Fibrocartilaginous Mesenchymoma...... 346 Bone-Forming Lesions that Simulate Neoplasms ...... 348 Heterotopic Ossification...... 348 Subungual Exostosis...... 350 Bizarre Parosteal Osteochondromatous Proliferation...... 352 Fracture Callus...... 354 Stress (Insufficiency) Fracture ...... 355 Bone Island...... 356 Miscellaneous Conditions that Simulate Neoplasm...... 357 Giant Cell Reparative Granuloma...... 357 Langerhans’ Cell Histiocytosis (Eosinophilic Granuloma)...... 358
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Erdheim-Chester Disease...... 361 Mast Cell Disease...... 363 Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease). . 365 Osteomyelitis...... 366 Paget’s Disease ...... 368 Hyperparathyroidism...... 370 Infarct of Bone...... 371 Transient Osteoporosis...... 373 Traumatic Osteolysis ...... 373 Chest Wall Hamartoma...... 373 Neuropathic Joint...... 375 Calcifying Pseudoneoplasm...... 376 15. Synovial Tumors...... 383 Pigmented Villonodular Synovitis...... 383 Malignant Pigmented Villonodular Synovitis...... 384 Synovial Chondromatosis ...... 386 Chondrosarcoma of Synovium ...... 389 Hemangioma ...... 390 Lipoma ...... 390 Index ...... 393
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