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Bones, Joints, and Soft-Tissue Tumors

Bones, Joints, and Soft-Tissue Tumors. Bone modeling, remodeling, and peak bone mass Osteoprogenitor cells Osteoblasts and lining cells Osteocytes - mechanotransduction Osteoclasts Proteins of bone matrix – Table 26-1

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Bones, Joints, and Soft-Tissue Tumors

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  1. Bones, Joints, and Soft-Tissue Tumors

  2. Bone modeling, remodeling, and peak bone mass • Osteoprogenitor cells • Osteoblasts and lining cells • Osteocytes - mechanotransduction • Osteoclasts • Proteins of bone matrix – Table 26-1 • Lamellar bone gradually replaces woven bone during growth and is deposited much more slowly and is stronger than woven bone Bones

  3. Bone Growth and Development • Homeobox genes • Primary center of ossification • Secondary center of ossification • Growth plate (physis) • Intramembranous formation • Appositional growth Bones

  4. Developmental Abnormalities in Bone Cells, Matrix, and Structure • Malformations and disease caused by defects in nuclear proteins and transcription factors • Diseases caused by defects in hormones and signal transduction mechanisms • Diseases associated with defects in extracellular structural proteins • Diseases associated with defects in folding and degradation of macromolecules • Diseases associated with defects in metabolic pathways ((enzymes, Ion channels, and transporters) • Diseases associated with decreased bone mass • Diseases caused by osteoclast dysfunction • Diseases associated with abnormal mineral homeostasis Bones

  5. OsteogenesisImperfecta • Brittle bone disease • Deficiency in the synthesis of type 1 collagen • Childhood fractures, blue sclera, hearing loss, dental abnormalities Bones

  6. Mucopolysaccharidoses • Abnormalities in hyaline cartilage • Short stature, chest wall abnormalities, malformed bones Bones

  7. Osteopetrosis • Marble bone disease ( Albers-Schonberg disease) • Reduced bone resorption and diffuse symmetric skeletal sclerosis • Impaired formation or function of osterclasts • Fractures, anemia, hydrocephaly • Cranial nerve defects • Infections Bones

  8. Osteoporosis • Porous bones and reduced bone mass • Categories of generalized osteoporosis • Table 26-4 • Pathophysiology of postmenopausal and senile osteoporosis – Figure 26-9 • Pathogenesis • Age-related changes • Reduced physical activity • Genetic factors • Calcium nutritional status • Hormonal influences • Clinical – vertebral fractures, bone density tests Bones

  9. Paget disease (osteitisdeformans) • Three phases • Initial osteolytic stage • Mixed osteoclastic-osteoblastic stage • Burnt-out quiescent osteosclerotic stage • Pathogenesis • Mosaic pattern of lamellar bone • Uncertain cause • Clinical • Elderly, often incidental finding, axial or proximal femur – 80%, pain heavy cranium, severe secondary osteoarthritis. tumors, fractures Bones

  10. Rickets in children = osteomalacia in adults • Hyperparathyroidism • Osteitisfibrosacystica = severe • Increased bone cell activity, peritrabecular fibrosis, cystic brown tumors Bones

  11. Renal Osteodystrophy • Increased osteoclastic bone resorption • Delayed matrix mineralization • Osteosclerosis • Growth retardation • Osteoporosis • Pathogenesis • CRF -> phosphate retention -> secondary hyperparathyroidism • Hypocalcemia develops because of decreased levels of 1,25 dihydroxy D3 • Increased osteoclast activity • Metabolic acidosis • Amyloid in bone from dialysis

  12. Fractures • Complete or incomplete • Closed (simple) • Compound • Comminuted • Displaced • Pathologic • Stress • Repair • Hematoma surrounds fracture-> creates framework-> soft-tissue callus -> bony callus -> reduced in size and shape until original outline is reestablished and medullary cavity is restored Bones

  13. Osteonecrosis ( Avascular necrosis) • Conditions associated with osteonecrosis • Table 26-5 • Trauma and corticosteroid use most common • Creeping substitution • Chronic pain, secondary arthritis Bones

  14. Infections – osteomyelitis • Pyogenic • Hematogeneous spread • Extension from a contiguous site • Direct implantaiton • Organisms • S.aureus 80-90% • Gram negatives • H.flu and GroupB strep in neonates • Salmonella in sickle cell anemia • TB – Pott disease – TB of the spine • Skeletal syphilis – nose, palate, skull, and extremities, “saber skin” Bones

  15. Bone tumor and tumor-like Lesions • Bone-forming tumors • Cartilage-forming tumors • Fibrous and fibro-osseous tumors • http://www.pediatricsconsultant360.com/Bone-Tumors-Childhood Bones

  16. Classification of Major Primary Tumors involving bones – Table 26-6 • Bone forming • Osteoma - sessile tumors, Gardner syndrome • Osteoid osteoma - < 2cm, nocturnal pain relieved by aspirin, teens and 20’s • Osteoblastoma - > 2 cm, pain not responsive to aspirin • Osteosarcoma – Malignant mesenchymal tumor, cancerous cells produce bone matrix, RB and p53, Bones

  17. Osteosarcoma • Most common primary malignant tumor of bone, exclusive of myeloma and lymphoma • Bimodal age distribution – 75% < 20 years of age, rest elderly with Paget, bone infarcts, proior irradiation • Figure 26-21 Major sites of origin • Subtypes • Site of origin • Degree of differentiation • Multicentricity • Primary or secondary • Histologic features • Clinical – Codman triangle, Painful, enlarging mass or pathologic fracture Bones

  18. Cartilage-forming tumors • Osteochondroma – exostosis, most common benign bone tumor, multiple hereditary exostosis syndrome, bones of endochondral origin, metaphysis near growth plate • Chondromas – endrochondromas, subperiosteal or juxtacortical, Ollier disease, Maffuci syndrome, most are incidental findings • Chondroblastoma – epiphysis or apophyses, painful, joint findings • Chondromyoxoid fibroma • Chondrosarcoma – intramedullary or juxtacortical (surface), conventional,clear cell, dedifferentiated, mesenchymal, central portion of the skeleton Bones

  19. Fibrous and fibro-osseous tumors • Fibrous cortical defect and non-ossifying fibroma • Fibrous dysplasia – monostotic or polyostotic without endocrine dysfuntion, poyostotic with café-au-lait spots and endocrinopathies ( McCune-Albright syndrome) • Fibrosarcoma variants Bones

  20. Miscellaneous Tumors • Ewing sarcoma/primitive neuroectodermal tumor – (11;22) (q24;q12) translocation, Homer-Wright rosettes • Giant-cell tumor – osteoclastoma • Aneurysmal bone cyst – multiloculated blood-filled cystic spaces • Metastatic disease – most common form of skeletal malignancy Bones

  21. Arthritis • Osteoarthritis • Rheumatoid arthritis • Juvenile idiopathic arthritis • Seronegativespondyloarthropathies • Infectious arthritis • Crystal-induced arthritis Joints

  22. Osteoarthritis • Degenerative joint disease • Progressive erosion of articular cartilage • Primary or secondary • Chondroytes, joint mice, bone eburnation • Deep,achy pain that worsens with use, morning stiffness, crepitus, limitation of ROM, hips, knees, lowerlumbar, cervical vertebrae, PIP And DIPs first carbometacarpal and first tarsometatarsal, Heberden nodes at DIP Joints

  23. Rheumatoid Arthritis • Chronic systemic inflammatory disorder that may affect many tissues and organs, - skin, blood vessels, heart, lungs, and muscle – but principally the joints, producing a nonsuppurative proliferative and inflammatory synovitis that often progresses to destruction of the articular cartilage and ankylosis of the joints • Pannus, fibrous ankylosis, rheumatoid nodules • HLA-DRB1 • Immunopathogenesis – Figure 26-44 • Symmetrical , small joints before large, warm,painful, stiff, joint effusions, juxtaarticular osteopenia, erosions and narrowing of joint space on x-ray, RF Joints

  24. Juvenile idiopathic arthritis • Classification • Systemic arthritis – fevers, fleeting ras, hepatosplenomegaly, serositis • Oligoarthritis – ANA+, iridoylitis • RF-positive polyarthritis • RF-negative polyarthritis • Enthesitis-assocaited arthritis – HLA-B27 • Psoriatic arthritis • Undifferentiated arthritis • Differs from adult RA • Oligoarthritis is more common • Systemic is more frequent • Large joints> small joints • RF and nodules absent • ANA+ Joints

  25. Seronegativespondyloarthropathies • Ankylosingspondyloarthritis – HLA-B27 • Reiter syndrome – with nongonococcal urethritis or cervitis and conjunctivits, HLA-B27, HIV • Enteritis-associated arthritis – GI infections • Psoriatic arthritis – HLA-B27 and HLA-Cw6 Joints

  26. Infectious Arthritis • Bacterial • TB • Lyme • Viral

  27. Crystal-Induced Arthritis • Gout and Gouty arthritis • Classification – Table 26-7 • Precipitation of monosodium urate crystals in the joints • Acute arthritis, chronic tophaceous arthritis, gouty nephropathy • Calcium pyrophosphate crystal deposition disease (pseudo-gout)

  28. Tumors and Tumor-like lesions • Ganglion and synovial cyst • Tenosynovial Giant-cell tumor (localized and diffuse) Joints

  29. Pathogenesis and general features – Table 26-8 • Fatty tumors • Fibrous tumors and tumor0like lesions • Fibrohistiocytic tumors • Tumors of skeletal muscle • Tumors of smooth muscle • Synovial sarcoma Soft-Tissue tumors and Tumor-Like Lesions

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