Specimens from the musculoskeletal system are infrequently encountered by the practicing pathologist. Many of the most common diagnoses, such as traumatic fractures, usually do not require tissue diagnosis. Furthermore, the rarity of bone and soft tissue neoplasms and the disfiguring surgery endorsed by a malignant diagnosis present unique challenges for the pathologist. The difficulty is compounded by the myriad diagnostic entities and the absence of universal classification systems. For the pathologist-in-training, learning the material has been equally demanding. The purpose of this textbook is to present the pathology of bone and soft tissue in a practical, focused, and easily accessible format. The text emphasizes the diagnostic hallmarks of each entity, to allow straightforward, rapid comparison of diagnoses within a single differential. More than 1000 illustrations are provided to supplement the critical details of the text. To achieve this format, discussion of minutiae and controversies is reduced.A\In most organ systems, non-neoplastic diseases constitute a significant proportion of diagnoses. Although this is also true of the skeletal system, the pathology of somatic soft tissue is dominated by neoplasms. The resulting disparity between the bone and soft tissue sections of this book, thus, reveals the reality of routine pathology practice rather than omission.A\Finally, although this book is intended for patholo-gists, diseases of bone cannot be diagnosed accurately without evaluation of corresponding radiographic studies. The fundamental radiographic findings for each diagnosis are described and illustrated with high-quality images so that the pathologist can effectively contribute to the multidisciplinary care of the patient.A\This volume would not have been possible without the hard work and dedication of the contributors. Thanks especially to Dr. Andrew Folpe and Dr. Carrie Inwards for their assistance and guidance. Andrew E. Horvai, MD, PhD; Thomas Link, MD

I.BONE AND JOINT

A.Metabolic Conditions, 2

      Paget Disease, 2

      Osteoporosis, 4

      Osteomalacia, 6

      Rickets, 8

      Osteogenesis Imperfecta, 10

      Hyperparathyroidism, 12

      Serous Fat Atrophy, 14

      Xanthomatosis, 15

      Osteopetrosis, 17

      Ochronosis. 19

      Melorheostosis, 21

      Gaucher Disease, 23

B.Infectious and Inflammatory Conditions, 25

      Osteoarthritis, 25

      Rheumatoid Arthritis, 28

      Septic Arthritis, 31

      Gout, 33

      Other Crystal-Induced Synovitis and Calcium Deposition, 35

      Acute Osteomyelitis, 38

      Chronic Osteomyelitis, 40

      Osteonecrosis, 43

      Bone Fracture and Fracture Callus, 45

C.Cystic Lesions, 47

      Aneurysmal Bone Cyst, 47

      Unicameral Bone Cyst, 50

      Intraosseous Ganglion, 52

D.Giant Cell-Rich Lesions, 54

      Giant Cell Tumor, 54

      Giant Cell-Reparative Granuloma, 57

      Brown Tumor, 59

      Tenosynovial Giant Cell Tumor, Localized Type, 60

      Tenosynovial Giant Cell Tumor, Diffuse Type, 62

E.Mixed PeRiosteal Lesions, 64

      Bizarre Parosteal Osteochondromatous Proliferation, 64

      Subungual Exostosis, 66

      Florid Reactive Periostitis, 68

F.Cartilage-Forming Lesions, 70

      Solitary Osteochondroma, 70

      Multiple Hereditary Exostoses, 73

      Enchondroma, 75

      Multiple Chondroma Syndromes [Oilier Disease, Maffucci Syndrome), 78

      Periosteal Chondroma, 80

      Synovial Chondromatosis, 82

      Chondro-Osseous Loose Body, 85

      Chondromyxoid Fibroma, 87

      Chondroblastoma, 89

      Conventional Intramedullary Chondrosarcoma, 92

      Chondrosarcoma of the Small Bones of the Hands and Feet, 96

      Periosteal Chondrosarcoma, 99

      Secondary Chondrosarcoma, 101

      Clear Cell Chondrosarcoma, 103

      Dediffercntiated Chondrosarcoma, 105

      Mesenchymal Chondrosarcoma, 108

G.Bone-Forming Lesions, 111

      Bone Island, 111

      Osteoid Osteoma, 112

      Osteoblastoma, 114

      Conventional Osteosarcoma, 116

      Small Cell Osteosarcoma, 119,

      Telangiectatic Osteosarcoma, 121

      High-Grade Surface Osteosarcoma, 123

      Post-Treatment Osteosarcoma, 125

      Parosteal Osteosarcoma, 127

      Periosteal Osteosarcoma, 130

      Low-Grade Central Osteosarcoma, 132

      Chondroblastoma-Like Osteosarcoma, 134

H. NOTOCHORDAL TUMORS, 136

      Benign Notochordal Cell Tumor, 136

      Chordoma, 138

I.Fibrous Lesions, 140

      Fibrous Dysplasia, 140

      Osteofibrous Dysplasia, 143

      Desmoplastic Fibroma, 145

      Nonossifying Fibroma, 147

      Ossifying Fibroma, 149

J.Vascular Lesions, 151

      Hemangioma, 151

      Synovial Hemangioma, 154

      Epithelioid Hemangioma, 156

      Epithelioid Hemangioendothelioma, 158

      Angiosarcoma of Bone, 160

      Massive Osteolysis, 163

K.EwING Sarcoma, 165

L.Hematopoietic Lesions, 168

      Primary Osseous Lymphoma, 168

      Acute Myeloid Leukemia, 170

      Plasmacytoma, 172

      Langerhans Cell Histiocytosis, 174

      Mastocytosis, 176

M.UNDIFFERENTIATED PlEOMORPHIC

      Sarcoma of Bone, 178

N.AdaMantinoma, 181

O.LipoMatous Tumors, 184

      Intraosseous Lipoma, 184

      Synovial Lipomatosis, 186

P.Carcinoma, 188

      Metastatic Carcinoma, 188

      Squamous Cell Carcinoma Arising in a Draining Sinus of Chronic Osteomyelitis, 191

II.SOFT TISSUE

A.Inflammatory and Pseudosarcomatous MYOFIBROBLASTiC PROLIFERATIONS, 194

      Nodular Fasciitis, 194

      Proliferative Myositis and Proliferative Fasciitis, 197

      Ischemic Fasciitis, 199

      Postoperative Spindle Cell Nodule, 201

      Necrotizing Fasciitis, 203

B.FiBrosing Lesions and Fibromatoses, 205

      Elastofibroma, 205

      Desmoplastic Fibroblastoma, 207

      Hypertrophic Scar, 209

      Keloid, 211

      Fibrous Hamartoma of Infancy, 212

      Gardner Fibroma, 214

      Nuchal Fibroma, 216

      Fibromatosis Colli, 217

      Calcifying Aponeurotic Fibroma, 219

      Fibroma of Tendon Sheath, 221

      Superficial Fibromatosis, 223

      Deep Fibromatosis, 225

      Inclusion-Body Fibroma, 228

      lgG4-Refated Sclerosing Disease, 230

C.Myofibroblastic Tumors, 232

      Myofibroblastoma (Mammary Type), 232

      Inflammatory Myofibroblastic Tumor, 234

      Myofibroma and Myofibromatosis, 237

      Intranodal Myofibroblastoma, 239

      Myofibroblastic Sarcoma, 241

D.Perivascular Tumors, 243

      Glomus Tumor, 243

      Glomuvenous Malformation, 245

      Myopericytoma, 246

E.flbroblastic and flbrohistiocytic Tumors, 248

      Reticulohistiocytoma, 248

      Juvenile Xanthogranuloma, 250

      Benign Fibrous Histiocytoma, 252

      Angiomatoid Fibrous Histiocytoma, 255

      Plexiform Fibrohistiocytic Tumor, 257

      Superficial Acral Fibromyxoma, 259

      Superficial Angiomyxoma, 261

      Dermatofibrosarcoma Protuberans, 262

      Giant Cell Fibroblastoma, 264

      Fibrosarcoma Arising in Dermatofibrosarcoma Protuberans, 266

      Adult Fibrosarcoma, 268

      Infantile Fibrosarcoma, 270

      Sclerosing Epithelioid Fibrosarcoma, 272

      Low-Grade Fibromyxoid Sarcoma, 273

      Atypical Fibroxanthoma, 275

      Intramuscular Myxoma, 277

      Myxofibrosarcoma, 279

F.Adipose Tumors, 281

      Lipoblastoma, 281

      Lipoma and Lipomatosis, 283

      Angiolipoma, 285

      Neural Fibrolipoma, 286

      Hibernoma, 287

      Chondroid Lipoma, 289

      Spindle Cell and Pleomorphic Lipoma, 290

      Well-Differentiated Uposarcoma and Atypical Lipomatous Tumor, 292

      Dedifferentiated Uposarcoma, 295

      Myxoid and Round Cell Uposarcoma, 298

      Pleomorphic Uposarcoma, 300

G.Smooth Muscle Tumors, 302

      Angioleiomyoma, 302

      Pilar Leiomyoma, 304

      Leiomyoma of Deep Soft Tissue, 305

      Leiomyosarcoma of Soft Tissue, 306

      Vascular Leiomyosarcoma, 309

      Epstein-Barr Virus-Associated Smooth Muscle Tumor, 311

H.Genital Stromal Tumors, 313

      Fibroepithelial Polyp, 313

      Angiomyofibroblastoma, 315

      Cellular Angiofibroma. 317

      Aggressive Angiomyxoma, 319

I.Skeletal Muscle Tumors, 321

      Adult Rhabdomyoma, 321

      Fetal Rhabdomyoma, 323

      Genital Rhabdomyoma, 324

      Alveolar Rhabdomyosarcoma, 325

      Embryonal Rhabdomyosarcoma, 327

      Pleomorphic Rhabdomyosarcoma, 330

J.Nerve and Nerve Sheath Tumors, 332

      Traumatic Neuroma, 332

      Schwannoma, 334

      Ancient Schwannoma, 336

      Ganglioneuroma, 338

      Neurothekeoma, 340

      Cellular Schwannoma, 343

      Neurofibroma, 345

      Plexiform Neurofibroma, 348

      Palisaded Encapsulated Neuroma, 350

      Perineurioma, 352

      Malignant Peripheral Nerve Sheath Tumor, 353

      Malignant Triton Tumor, 356

      Granular Cell Tumor, 358

      Malignant Granular Cell Tumor, 360

      Neuroglial Heterotopia, 362

      Neuroblastoma, 364

      Esthesioneuroblastoma, 366

      Paraganglioma, 368

      Melanotic Neuroectodermal Tumor of Infancy, 370

K.Vascular Tumors, 372

      Papillary Endothelial Hyperplasia, 372

      Pyogenic Granuloma, 374

      Arteriovenous Malformation, 376

      Capillary Hemangioma, 378

      Cavernous Hemangioma, 380

      Lymphangioma, 382

      Epithelioid Hemangioma, 384

      Kimura Disease, 385

      Spindle Cell Hemangioma, 387

      Sinusoidal Hemangioma, 389

      Epithelioid Hemangioendothelioma, 390

      Hobnail Hemangioendothelioma, 393

      Angiosarcoma, 395

      Intimal Sarcoma, 398

      Kaposi Sarcoma, 400

      Kaposiform Hemangioendothelioma, 403

L.Bone and Cartilage Tumors of Soft Tissue, 405

      Soft Tissue Chondroma, 405

      Extraskeletal Myxoid Chondrosarcoma, 407

      Nuchal Fibrocartilaginous Pseudotumor, 409

      Myositis Ossificans, 411

      Ossifying Fibromyxoid Tumor, 414

      Soft Tissue Osteosarcoma, 417

M.PerIVascular Epithelioid Cell Neoplasms, 419

N.Tumors of Uncertain Lineage, 421

      Pleomorphic Hyalinizing Angiectatic Tumor of Soft Parts, 421

      Soft Tissue Myoepithelioma, 423

      Alveolar Soft Part Sarcoma, 426

      Clear Cell Sarcoma, 428

      Synovial Sarcoma, 430

      Extrarenal Rhabdoid Tumor, 433

      Epithelioid Sarcoma (Proximal and Distal), 435

      Desmoplastic Small Round Cell Tumor, 438

      Primitive Neuroectodermal Tumor (Ewing Sarcoma), 440

      Giant Cell Tumor of Soft Tissue, 442

      Undifferentiated Pleomorphic Sarcoma, 444

      Phosphaturic Mesenchymal Tumor, 446

      Myxoinflammatory Fibroblastic Sarcoma, 448

      Ectopic Hamartomatous Thymoma, 451

      Solitary Fibrous Tumor and Hemangiopericytoma, 453

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