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78 Cards in this Set
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osteoporosis
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Metabolic bone disease, decrease bone mass, 8F>1M, more whites
Same osteoid: mineralized bone but thinner cortex and less coarse cancellous bone trabeculae. Seen most in cancellous bone (vertebrae), causes wedging and collapse. Type 1: disruption in connections between trabeculae → fx. Post-menopausal women, increase in osteoclastic activity due to estrogen withdrawal → IL-1, Il-6, TNF, MCSF) Type 2 (senile osteoporosis): fewer trabecular bone, attenuated osteoblastic function can’t keep up with osteoclasts. Factors: genetics (bone mass, BMD), calcium intake, calcium absorption and vitamin D (less 1 alpha hydroxylase activity, decreased kidney response to PTH), exercise, cigarettes (decreased estrogen) |
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osteogenesis imperfecta
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AD, type 1 collagen (chr 7 and 17), 4 types. Affects skeleton, joints, ears, ligaments, teeth, sclerae, skin
Rad: osteopenia, variable deformity. Histo: disorganized growth plate, no trabecular pattern, numbreous plump osteocytes and prominent osteoid seams OI 1: mildest (AD), fx as toddler, blue sclera, hearing and teeth abnormality, flat feet, kyphoscoliosis Histo: thing bones, exuberant callus formation. OI 2: lethal, prenatal (AD) OI 3: progressive, many bone fxs, growth retardation, severe skeletal abn. Sclerae become white. Teeth abn. OI 4: like type 1 but with normal sclerae, cortical bone: woven bone with some lamellar bone, fractures (treat aggressively to allow growth) |
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multiple endchondromatosis
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osteonecrosis
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death of bone and marrow in the absence of infection: caused by trauma, emboli, steroids, radiation, organ transplantation<br>Histo: cancellous bone is replaced by creeping substitution, creeping neovascular tissue replaces marrow. cortical bone is replaced by cutting cones (along preexisting channels), osteoclasts and osteoblasts enter to make new bone.<br>Rad: dense due to osteoporosis in surrounding bone compared to necrotic bone, addition of new bone (creeping substitution), formation of calcium soaps, compaction of necrotic bone
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reactive bone formation
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myositis ossificans circumscripta
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chronic osteomyelitis
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inflammation of the bone and bone marrow, usually bacterial (Staph, also E. coli, N. gonorrhea, H. flu, Salmonella).
Direct penetration: bacteria enter via wounds, fractures, surgical procedures Hematogenous: from blood stream, boys 5-15yo, drug addicts. Open growth plate → vascular loop system → slow blood allows bacteria to get in → proliferation → pressure on bone → bone resorption If not contained, pus and bacteria can enter cortex and periosteum, shear off parosteal blood vessels and even eventually involve the joint. Chronic: lymphocytes and plasma cells, marrow fibrosis Comps: septicemia, acute bacterial arthritis, pathologic fx, chronic osteomyelitis, SCC, amyloidosis. |
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TB
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congenital syphillis
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eosinophillic granuloma
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osteoporosis
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Metabolic bone disease, decrease bone mass, 8F>1M, more whites
Same osteoid: mineralized bone but thinner cortex and less coarse cancellous bone trabeculae. Seen most in cancellous bone (vertebrae), causes wedging and collapse. Type 1: disruption in connections between trabeculae → fx. Post-menopausal women, increase in osteoclastic activity due to estrogen withdrawal → IL-1, Il-6, TNF, MCSF) Type 2 (senile osteoporosis): fewer trabecular bone, attenuated osteoblastic function can’t keep up with osteoclasts. Factors: genetics (bone mass, BMD), calcium intake, calcium absorption and vitamin D (less 1 alpha hydroxylase activity, decreased kidney response to PTH), exercise, cigarettes (decreased estrogen) |
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osteomalacia
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Metabolic bone disease
Osteomalacia: adults, inadequate mineralization of newly formed bone matrix Rickets: kids, extensive changes within the growth plate (prolif and calcified cartilage zones continue to grow, becomes lobulated and irregular with endochondral calcification. Assoc with abnormal vit D, phosphate deficiency, defects in mineralization. Rad: osteopenia, compression fractures, Pseudofractures of Milkman-Looser (radiolucent, transverse defects, no associated callus formation) Histo: exaggeration of osteoid seams in thicenss and proportion of trabecular surface covered. Clin: muscle weakness, difuse aches and pain, or poorly localized bone pain and tenderness. Kids: apathetic, irritable, short attention span, bad bones and teeth, frontal bossing, rachitis rosary, pectus carinatum. |
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primary hyperparathyroid
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Metabolic bone disease
PTH → increased resorption by accelerated remodeling. 90% due to adenomas, 10% due to 4gland hyperplasias. 3 stages: early (osteoclast stimulation, cutting cones, peritrabecular fibrosis), Osteitis fibrosa (loose marrow fibrosis, hemosiderin-laden macrophages, hemorrhage from microfx and reactive woven bone), osteitis fibrosa cystica (cystic degeneration, gian cells, reactive bone, hemosiderin-laden macrophages. “Brown tumors” : can look like giant cell tumors, check PTH! |
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primary hyperparathyroid
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Metabolic bone disease
PTH → increased resorption by accelerated remodeling. 90% due to adenomas, 10% due to 4gland hyperplasias. 3 stages: early (osteoclast stimulation, cutting cones, peritrabecular fibrosis), Osteitis fibrosa (loose marrow fibrosis, hemosiderin-laden macrophages, hemorrhage from microfx and reactive woven bone), osteitis fibrosa cystica (cystic degeneration, gian cells, reactive bone, hemosiderin-laden macrophages. “Brown tumors” : can look like giant cell tumors, check PTH! |
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renal osteodystrophy
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fibrosis on A, osteomalacia on B and adynamic bone disease on C
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Paget's dz
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Disordered bone remodeling. >60yo, only one or a few bones. Hereditary and viral origins proposed.
Histo: 3 stages: hot (osteoclastic resorptive stage) (lytic lesions in the cortex, osteolysis, mild fibrosis, dilated marrow sinusoids). Mixed stage (osteblasts and osteoclasts, thickened cortical and cancellous bone, abundant but haphazard cellular activity). Cold (burnt out) stage (little cellular activity, disordered thickened bones, osteoclasts with >100 nuclei (killer clasts), prominent mosaic pattern of trabecular bone with paratrabecular fibrosis. Clin: Pain, axial>appendicular, skull , pagetic steal (light-headedness due to increased blood demand), fx, arthritis, high-output cardiac failure, sarcomatous change (<1%), severe disease. Increased Alk Phos. Most pts asx and require no treatment. Calcitonin, bisphosphonates, mithramycin can be used. |
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Paget's Dz
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Disordered bone remodeling. >60yo, only one or a few bones. Hereditary and viral origins proposed.
Histo: 3 stages: hot (osteoclastic resorptive stage) (lytic lesions in the cortex, osteolysis, mild fibrosis, dilated marrow sinusoids). Mixed stage (osteblasts and osteoclasts, thickened cortical and cancellous bone, abundant but haphazard cellular activity). Cold (burnt out) stage (little cellular activity, disordered thickened bones, osteoclasts with >100 nuclei (killer clasts), prominent mosaic pattern of trabecular bone with paratrabecular fibrosis. Clin: Pain, axial>appendicular, skull , pagetic steal (light-headedness due to increased blood demand), fx, arthritis, high-output cardiac failure, sarcomatous change (<1%), severe disease. Increased Alk Phos. Most pts asx and require no treatment. Calcitonin, bisphosphonates, mithramycin can be used. |
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fibrous dysplasia
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Developmental disorder, fibrous and osseous elements<br>Rad: ground glass lesions, well-marginated<br>Monostotic or Polyostotic. May be quiescent at puberty, may be stimulated by pregnancy.
Tx: Repair of fractures. Pathogenesis: increased cAMP from genetic mutations Histo: benign fibroblastic tissue in loose whorled pattern, irregular spicules, no osteoblastic rims. 10% have islands of cartilage, can have cystic degeneration with hemosiderin-laden macrophages, hemorrhage, osteoclasts |
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solitary bone cyst
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benign, fluid-filled, unlilocular lesions
M>F Metaphyseal, by growth plate, proximal humerus/femur Disturbed bone growth by trauma, maybe due to hematoma, pressure → resorption of bone. Slow process. Rad: well-marginated radiolucent (lytic) lesion Histo: thing fibrous tissue, few giant cells and hemosiderin-laden macrophages, chronic inflammatory cells, reactive bone lining cyst. Osteoclasts on advancing edge. Proteinaceous material in cysts. |
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aneurysmal bone cyst
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uncommon, hyperemic, children and young adults, history of trauma
long bones and vertebrae rad: lytic lesions on x-ray, fluid-fluid levels on MRI rupture and hemorrhage is possible gross: ballooned periosteum (blood-filled sponge) histo: granulation tissue, scattered multinucleated giant cells, osteoid trabeculae |
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osteoid osteoma
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small painful lesion of osseous tissue (nidus) surrounded by halo of reacive bone formation
5-25yo diaphysis, cortex of long bones, esp lower limbs clin: pain (nocturnal, worse with EtOH, better with aspirin) rad: sclerotic ortical based lesion with central nidus, <1cm gross: spherical, hyperemic, soft. Soaks up tetracycline histo: cellular, granulation tissue, osteoblasts, osteoclasts, thing trabecular bone more mature at the center with sclerotic reactive bone around tx: curettage, elcrocautery, radio frequency ablation, mini-bloc excision |
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chondroblastoma
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uncommon, benign. Epiphyseal of tubber femur, tibia and humerus
5-25yo, M>F moderate pain, larger lesions can destroy the bone rad: eccentric, radiolucent lesion, sharp borders. Mottled matrix on CT goss: soft, compact, scattered gray or hemorrhagic area. Histo: primite chondroblasts arranged in sheet of round/polyhedral cells with grooved nuclei. Calcification, multinucleated giant cells. Tx: curettage, 10% recur. |
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osteosarcoma
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most common primary bone tumor
10-20yo, M>F, knee or shoulder, metaphyseal. Older ppl, it’s due to Paget or radiation. RB and p53 mutations Clin: mild/intermittent pain, swelling, tenderness, increased serum Alk Phos Rad: lytic lesions, periosteum with rim of reactive bone (Codman’s triangle (uplifted periosteum), sunburst pattern) Gross: variable Histo: malignant osteoblasts produce woven bone, haphazard, foci of malignant cartilage, pleomorphic giant cells. Atypical mitotic figures. Tx: adjuvant therapy, limb sparing surgery. 85% 5yr survival. Mets to lungs, also to bones, pleura, heart. |
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giant cell tumor
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locally aggressive, can be malignant, more in Asian countries, F>M, microfx.
rad: between metaphysis and epiphysis of long bones. Lytic lesion with little periosteal reaction. Thin bony shell, may extend into bone. Soap bubble. Gross: hemorrhagic area, blood-filled sponge histo: Mononuclear stroma (plump, large nucleus, mitoses), large osteoclastic multinucleated cells (random distribution). Rich vascularized stroma. Tx: curettage, bone grafting, mets to lungs (5-10%) |
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ewing sarcoma
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uncommon, malignant, M>F, rare in AA, small round cells
from primitive marrow elements , t(11;22) (worse prog) May have fever and weakness, bone pain rad: midshaft/diaphysis, onion-skin pattern (periosteal new bone reaction to keep in check). Gross: soft and grayish, hemorrhage and focal necrosis, soft tissue expansion Histo: sheets of packed small round cells, irregular nests of cells, little/no interstitial stroma, PAS positive, CD99 positive Tx: chemo, radiation, surgery. 60-75% 5yr survival |
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multiple myeloma
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malignant plasma cell tumor, older people, M>F
rad: lytic due to cytokines, especially skull, spine, ribs, pelvis, femur histo: sheets of plasma cells with varying maturity, amyloid in 10%, SPEP for monoclonal process tx: chemotherapy, radiation cause of death: kidney failure |
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metastatic carcinoma
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most common malignant bone tumor
breast, prostate, lung, thyroid, kidney. Vertebral column is the most common. thyroid, kidney, neuroblastoma and GI are lytic; prostate, breast, lung and stomach are blastic. Usually have a mix of lytic and blastic |
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articular hyaline cartilage
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ankylosing spondylitis
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CPPD
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pigmented villonodular synovitis
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nodular fasciitis
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nodular fasciitis
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benign, rapidly-growing, from trauma.
Supl tissues of forearm, trunk, and back, usually in adults on extensor surfaces. Histo: cellular mitotically active lesion with pleomarphic spindle cells. Exuberant granulation tissue. Tx: excision |
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fibrosarcoma
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malignant tumor of fibrobalsts, usually I thigh, adult, any age.
Congenital: t(12;15) Gross: firm, sharply demarcated, necrosis and hemorrhage Histo: pleomorphic fibroblasts in dense interlacing bundles and fascicles (herringbone pattern). Bad prognosis |
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pleomorphic spindle cell sarcoma
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liposarcoma
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malignant, second most common
50yo, deep thigh, retroperitoneum, slow-growing. Myxoid variant: t(12;16) gross: 5-10cm, may have necrosis, cysts, hemorrhage histo: lipoblasts (signet ring) in a vascularized myxoid stroma tx: prog depends on differentiation |
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rhabdomyosarcoma
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children and young adults, from primitive mesenchyme
embryonal rhabdomyosarcoma: most common type, 3-12yo, usually head and neck. Immunohistochemical stains make the diagnosis. Molecular studies for alveolar type: t(2;13) (bad prognosis). Botryoid and pleomorphic types also exist. Tx: surgery, chemo, radiation. |
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synovial sarcoma
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highly malignant, comes from tendon sheaths, bursa in joint capsules, <10% interarticular.
Adolescants and young adults in large joints. T(x;18) Gross: circumscribed, round or multilobular mass. Pseudocapsule, cystic changes. Histo: spindle cell and epithelia-like area. Monophasic (spindled) variant also exists. Immunohistochemical stains for epithelial differentiation Tx: high recurrence, mets in >60%, 50% to lung mets. |
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Osteopetrosis
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inheritable; abnormally dense bone
mortality from anemia, CN entrapment, hydrocephalus, infection (AR) AD: more benign, no sxs. Decreased osteoclast function (TRIRG1 gene, carbonic anhydrase gene) x-ray: short opaque bones clin: bone weakness, fractures, anemia, hypopenia, deafness, blindness gross: erlenmeyer flask bone, thickened cortex, no metaphyseal funnelization histo: irregular bone tissue with cartilage, osteoclasts increased, decreased or normal. tx: bone-marrow transplant |
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Osteogenesis imperfecta
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AD, type 1 collagen (chr 7 and 17), 4 types. Affects skeleton, joints, ears, ligaments, teeth, sclerae, skin
Rad: osteopenia, variable deformity. Histo: disorganized growth plate, no trabecular pattern, numbreous plump osteocytes and prominent osteoid seams OI 1: mildest (AD), fx as toddler, blue sclera, hearing and teeth abnormality, flat feet, kyphoscoliosis Histo: thing bones, exuberant callus formation. OI 2: lethal, prenatal (AD) OI 3: progressive, many bone fxs, growth retardation, severe skeletal abn. Sclerae become white. Teeth abn. OI 4: like type 1 but with normal sclerae, cortical bone: woven bone with some lamellar bone, fractures (treat aggressively to allow growth) |
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Osteonecrosis
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death of bone and marrow in the absence of infection: caused by trauma, emboli, steroids, radiation, organ transplantation
Legg-Calve-Perthes disease: focal bone necrosis involving the femoral head in children→ stress fxs, compaction, neovascularization, subchondral cracking → collapse of subchondral bone → severe arthritis Due to open growth plate in kids that don’t allow blood vessels through → fed by blood vessels around neck, susceptible to trauma and compression Histo: cancellous bone is replaced by creeping substitution, creeping neovascular tissue replaces marrow. cortical bone is replaced by cutting cones (along preexisting channels), osteoclasts and osteoblasts enter to make new bone. Rad: dense due to osteoporosis in surrounding bone compared to necrotic bone, addition of new bone (creeping substitution), formation of calcium soaps, compaction of necrotic bone. |
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Osteomyelitis
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inflammation of the bone and bone marrow, usually bacterial (Staph, also E. coli, N. gonorrhea, H. flu, Salmonella).
Direct penetration: bacteria enter via wounds, fractures, surgical procedures Hematogenous: from blood stream, boys 5-15yo, drug addicts. Open growth plate → vascular loop system → slow blood allows bacteria to get in → proliferation → pressure on bone → bone resorption If not contained, pus and bacteria can enter cortex and periosteum, shear off parosteal blood vessels and even eventually involve the joint. Open sinus tract can go to skin |
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Osteomyelitis
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inflammation of the bone and bone marrow, usually bacterial (Staph, also E. coli, N. gonorrhea, H. flu, Salmonella).
Direct penetration: bacteria enter via wounds, fractures, surgical procedures Hematogenous: from blood stream, boys 5-15yo, drug addicts. Open growth plate → vascular loop system → slow blood allows bacteria to get in → proliferation → pressure on bone → bone resorption If not contained, pus and bacteria can enter cortex and periosteum, shear off parosteal blood vessels and even eventually involve the joint. Open sinus tract can go to skin |
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Acute osteomyelitis
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PMN infiltrate, nonviable trabecular bone and marrow
Comps: septicemia, acute bacterial arthritis, pathologic fx, chronic osteomyelitis, SCC, amyloidosis. |
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Chronic osteomyelitis
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lymphocytes and plasma cells, marrow fibrosis
Comps: septicemia, acute bacterial arthritis, pathologic fx, chronic osteomyelitis, SCC, amyloidosis. |
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Osteoporosis
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Metabolic bone disease, decrease bone mass, 8F>1M, more whites
Same osteoid: mineralized bone but thinner cortex and less coarse cancellous bone trabeculae. Seen most in cancellous bone (vertebrae), causes wedging and collapse. Type 1: disruption in connections between trabeculae → fx. Post-menopausal women, increase in osteoclastic activity due to estrogen withdrawal → IL-1, Il-6, TNF, MCSF) Type 2 (senile osteoporosis): fewer trabecular bone, attenuated osteoblastic function can’t keep up with osteoclasts. Factors: genetics (bone mass, BMD), calcium intake, calcium absorption and vitamin D (less 1 alpha hydroxylase activity, decreased kidney response to PTH), exercise, cigarettes (decreased estrogen) |
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Primary HyperPTH
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Metabolic bone disease
PTH → increased resorption by accelerated remodeling. 90% due to adenomas, 10% due to 4gland hyperplasias. 3 stages: early (osteoclast stimulation, cutting cones, peritrabecular fibrosis), Osteitis fibrosa (loose marrow fibrosis, hemosiderin-laden macrophages, hemorrhage from microfx and reactive woven bone), osteitis fibrosa cystica (cystic degeneration, gian cells, reactive bone, hemosiderin-laden macrophages. “Brown tumors” : can look like giant cell tumors, check PTH! |
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Osteomalacia/Rickets
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Metabolic bone disease
Osteomalacia: adults, inadequate mineralization of newly formed bone matrix Rickets: kids, extensive changes within the growth plate (prolif and calcified cartilage zones continue to grow, becomes lobulated and irregular with endochondral calcification. Assoc with abnormal vit D, phosphate deficiency, defects in mineralization. Rad: osteopenia, compression fractures, Pseudofractures of Milkman-Looser (radiolucent, transverse defects, no associated callus formation) Histo: exaggeration of osteoid seams in thickness and proportion of trabecular surface covered. Clin: muscle weakness, difuse aches and pain, or poorly localized bone pain and tenderness. Kids: apathetic, irritable, short attention span, bad bones and teeth, frontal bossing, rachitis rosary, pectus carinatum |
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Paget's disease
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Disordered bone remodeling. >60yo, only one or a few bones. Hereditary and viral origins proposed.
Histo: 3 stages: hot (osteoclastic resorptive stage) (lytic lesions in the cortex, osteolysis, mild fibrosis, dilated marrow sinusoids). Mixed stage (osteblasts and osteoclasts, thickened cortical and cancellous bone, abundant but haphazard cellular activity). Cold (burnt out) stage (little cellular activity, disordered thickened bones, osteoclasts with >100 nuclei (killer clasts), prominent mosaic pattern of trabecular bone with paratrabecular fibrosis. Clin: Pain, axial>appendicular, skull , pagetic steal (light-headedness due to increased blood demand), fx, arthritis, high-output cardiac failure, sarcomatous change (<1%), severe disease. Increased Alk Phos. Most pts asx and require no treatment. Calcitonin, bisphosphonates, mithramycin can be used. |
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Tuberculosis (Pott's disease)
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From focus elsewhere, affects the vertebral bodies, sparing lamina and spines
Usually thoracic > T11, cervical or lumbar vertebral body collapses histo: caseating granulomas, inflimmation, slow resorption of bone, little or no woven bone collapse special studies: AFB Cold abscess, psoas abscess |
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Fibrous dysplasia
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Developmental disorder, fibrous and osseous elements
Rad: ground glass lesions, well-marginated Monostotic: most common, 2nd/3rd decade, proximal femus, tibia, ribs, facial bones. Asymptomatic or fractures Polyostotic: F>M, more than half of the skeleton is affected. Presents in childhood with fractures. May be quiescent at puberty, may be stimulated by pregnancy. Tx: Repair of fractures. Pathogenesis: increased cAMP from genetic mutations Histo: benign fibroblastic tissue in loose whorled pattern, irregular spicules, no osteoblastic rims. 10% have islands of cartilage, can have cystic degeneration with hemosiderin-laden macrophages, hemorrhage, osteoclasts <1% become sarcomas |
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Non-ossifying fibroma
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Metaphysis
25% of kids 4-10yo, usually regresses, usually asx. Rad: cortical, eccentric, well-demarcated, central lucent zone with scalloped scleroti margins Gross: granular, dark red or brown Histo: bland spindle cells, interlacing whorled pattern, multinucleated giant cells and foamy macrophages Tx: curettage, bone grafting |
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Solitary bone cyst
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benign, fluid-filled, unlilocular lesions
M>F Metaphyseal, by growth plate, proximal humerus/femur Disturbed bone growth by trauma, maybe due to hematoma, pressure → resorption of bone. Slow process. Rad: well-marginated radiolucent (lytic) lesion Histo: thing fibrous tissue, few giant cells and hemosiderin-laden macrophages, chronic inflammatory cells, reactive bone lining cyst. Osteoclasts on advancing edge. Proteinaceous material in cysts. |
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Aneurysmal bone cyst
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uncommon, hyperemic, children and young adults, history of trauma
long bones and vertebrae rad: lytic lesions on x-ray, fluid-fluid levels on MRI rupture and hemorrhage is possible gross: ballooned periosteum (blood-filled sponge) histo: granulation tissue, scattered multinucleated giant cells, osteoid trabeculae |
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Osteoid osteoma
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small painful lesion of osseous tissue (nidus) surrounded by halo of reacive bone formation
5-25yo diaphysis, cortex of long bones, esp lower limbs clin: pain (nocturnal, worse with EtOH, better with aspirin) rad: sclerotic ortical based lesion with central nidus, <1cm gross: spherical, hyperemic, soft. Soaks up tetracycline histo: cellular, granulation tissue, osteoblasts, osteoclasts, thing trabecular bone more mature at the center with sclerotic reactive bone around tx: curettage, elcrocautery, radio frequency ablation, mini-bloc excision |
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Osteoid osteoma
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small painful lesion of osseous tissue (nidus) surrounded by halo of reacive bone formation
5-25yo diaphysis, cortex of long bones, esp lower limbs clin: pain (nocturnal, worse with EtOH, better with aspirin) rad: sclerotic ortical based lesion with central nidus, <1cm gross: spherical, hyperemic, soft. Soaks up tetracycline histo: cellular, granulation tissue, osteoblasts, osteoclasts, thing trabecular bone more mature at the center with sclerotic reactive bone around tx: curettage, elcrocautery, radio frequency ablation, mini-bloc excision |
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Osteoblastoma
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similar to osteoid osteoma but larger, without nocturnal pain relieved by aspirin
spine and long bones, 10-35yo, M=F. tx: curettage |
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Enchondroma
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may be a hamartoma, but is neoplastic
asny age, asx, usually small bones of hands and feet. Small and slow-growing. Rad: well-delineated radiolucent areas, stippled calcification Gross: semi-translucent, few calcified area Histo: well-differentiated hyaline cartilage, sparsely cellular, minimal cellular pleomorphism (except maybe hands and feet) Tx: curettage |
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Enchondroma
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may be a hamartoma, but is neoplastic
asny age, asx, usually small bones of hands and feet. Small and slow-growing. Rad: well-delineated radiolucent areas, stippled calcification Gross: semi-translucent, few calcified area Histo: well-differentiated hyaline cartilage, sparsely cellular, minimal cellular pleomorphism (except maybe hands and feet) Tx: curettage |
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Chondroblastoma
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uncommon, benign. Epiphyseal of tubber femur, tibia and humerus
5-25yo, M>F moderate pain, larger lesions can destroy the bone rad: eccentric, radiolucent lesion, sharp borders. Mottled matrix on CT goss: soft, compact, scattered gray or hemorrhagic area. Histo: primite chondroblasts arranged in sheet of round/polyhedral cells with grooved nuclei. Calcification, multinucleated giant cells. Tx: curettage, 10% recur. |
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Osteosarcoma
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most common primary bone tumor
10-20yo, M>F, knee or shoulder, metaphyseal. Older ppl, it’s due to Paget or radiation. RB and p53 mutations Clin: mild/intermittent pain, swelling, tenderness, increased serum Alk Phos Rad: lytic lesions, periosteum with rim of reactive bone (Codman’s triangle (uplifted periosteum), sunburst pattern) Gross: variable Histo: malignant osteoblasts produce woven bone, haphazard, foci of malignant cartilage, pleomorphic giant cells. Atypical mitotic figures. Tx: adjuvant therapy, limb sparing surgery. 85% 5yr survival. Mets to lungs, also to bones, pleura, heart. |
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Chondrosarcoma
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second most common primary malignant tumor
4-6th decades, M>F clin: deep pain, usually central (pelvic, ribs, etc) rad: lobulated, lytic mass with stippled/ring-like calcifications histo: grading is important (1: well-differentiated (may look like enchondroma), 3- poorly differentiated). Rearrangement of chr17, t(9;22) in extraskeletal tx: prog good for well-differentiated, less for high grade. Wide surgical excision |
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Chondrosarcoma
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second most common primary malignant tumor
4-6th decades, M>F clin: deep pain, usually central (pelvic, ribs, etc) rad: lobulated, lytic mass with stippled/ring-like calcifications histo: grading is important (1: well-differentiated (may look like enchondroma), 3- poorly differentiated). Rearrangement of chr17, t(9;22) in extraskeletal tx: prog good for well-differentiated, less for high grade. Wide surgical excision |
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Ewing sarcoma
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uncommon, malignant, M>F, rare in AA, small round cells
from primitive marrow elements , t(11;22) (worse prog) May have fever and weakness, bone pain rad: midshaft/diaphysis, onion-skin pattern (periosteal new bone reaction to keep in check). Gross: soft and grayish, hemorrhage and focal necrosis, soft tissue expansion Histo: sheets of packed small round cells, irregular nests of cells, little/no interstitial stroma, PAS positive, CD99 positive Tx: chemo, radiation, surgery. 60-75% 5yr survival |
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Giant cell tumor of bone
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locally aggressive, can be malignant, more in Asian countries, F>M, microfx.
rad: between metaphysis and epiphysis of long bones. Lytic lesion with little periosteal reaction. Thin bony shell, may extend into bone. Soap bubble. Gross: hemorrhagic area, blood-filled sponge histo: Mononuclear stroma (plump, large nucleus, mitoses), large osteoclastic multinucleated cells (random distribution). Rich vascularized stroma. Tx: curettage, bone grafting, mets to lungs (5-10%) |
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Multiple myeloma
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malignant plasma cell tumor, older people, M>F
rad: lytic due to cytokines, especially skull, spine, ribs, pelvis, femur histo: sheets of plasma cells with varying maturity, amyloid in 10%, SPEP for monoclonal process tx: chemotherapy, radiation cause of death: kidney failure |
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metastatic tumors
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most common malignant bone tumor
breast, prostate, lung, thyroid, kidney. Vertebral column is the most common. thyroid, kidney, neuroblastoma and GI are lytic; prostate, breast, lung and stomach are blastic. Usually have a mix of lytic and blastic |
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soft tissue
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Nodular fascitis
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benign, rapidly-growing, from trauma.
Supl tissues of forearm, trunk, and back, usually in adults on extensor surfaces. Histo: cellular mitotically active lesion with pleomarphic spindle cells. Exuberant granulation tissue. Tx: excision |
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soft tissue
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fibromatosis
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locally invasive, slow-growing, don’t metastasize but recurr
risk: riabetics, alcoholics, epileptics gross: large, whitish, poorly demarcated histo: bland spindle cells (no pleoorphism or mitoses). Palmar (dupuytren) (most common), plantar, penile (peyronie) |
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soft tissue
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fibrosarcoma
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malignant tumor of fibrobalsts, usually I thigh, adult, any age.
Congenital: t(12;15) Gross: firm, sharply demarcated, necrosis and hemorrhage Histo: pleomorphic fibroblasts in dense interlacing bundles and fascicles (herringbone pattern). Bad prognosis |
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soft tissue
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Malignant fibrous histiocytoma
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most common sarcoma, due to radiation
older adults, deep fascia/skeletal muscle histo: spindle shaped tumor cells, pleomorphism and mitotic figures. Histiocytic (macrophage) differentiation) tx: 50% recur, mets to lungs, excision. |
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soft tissue
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lipoma
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most common soft tissue mass (benign)
gross: yellow, soft and lobulated histo: well-differentiated adipocytes, no atypia or fibroblasts |
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soft tissue
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liposarcoma
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malignant, second most common
50yo, deep thigh, retroperitoneum, slow-growing. Myxoid variant: t(12;16) gross: 5-10cm, may have necrosis, cysts, hemorrhage histo: lipoblasts (signet ring) in a vascularized myxoid stroma tx: prog depends on differentiation |
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soft tissue
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leiomyoma
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benign smooth muscle tumor, subcutaneous or vessel wall
gross: painful, firm yellow nodule histo: bland spindle cells, low mitotic rate |
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soft tissue
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leiomyosarcoma
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malignant soft tissue tumor
gross: foci of necrosis, hemorrhage, cystic degeneration histo: fascicle of spindle cells, cellular atypia, mitotic rate, mitotic rate → prognosis tx: surgery, adjuvant therapy if high-grade. Most eventually metastasize. |
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soft tissue
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rhabdomyosarcoma
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children and young adults, from primitive mesenchyme
embryonal rhabdomyosarcoma: most common type, 3-12yo, usually head and neck. Immunohistochemical stains make the diagnosis. Molecular studies for alveolar type: t(2;13) (bad prognosis). Botryoid and pleomorphic types also exist. Tx: surgery, chemo, radiation. |
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soft tissue
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rhabdomyosarcoma
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children and young adults, from primitive mesenchyme
embryonal rhabdomyosarcoma: most common type, 3-12yo, usually head and neck. Immunohistochemical stains make the diagnosis. Molecular studies for alveolar type: t(2;13) (bad prognosis). Botryoid and pleomorphic types also exist. Tx: surgery, chemo, radiation. |
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soft tissue
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synovial sarcoma
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highly malignant, comes from tendon sheaths, bursa in joint capsules, <10% interarticular.
Adolescants and young adults in large joints. T(x;18) Gross: circumscribed, round or multilobular mass. Pseudocapsule, cystic changes. Histo: spindle cell and epithelia-like area. Monophasic (spindled) variant also exists. Immunohistochemical stains for epithelial differentiation Tx: high recurrence, mets in >60%, 50% to lung mets. |