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78 Cards in this Set

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osteoporosis
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)
osteogenesis imperfecta
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)
multiple endchondromatosis
osteonecrosis
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
reactive bone formation
myositis ossificans circumscripta
chronic osteomyelitis
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.
TB
congenital syphillis
eosinophillic granuloma
osteoporosis
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)
osteomalacia
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.
primary hyperparathyroid
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!
primary hyperparathyroid
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!
renal osteodystrophy
fibrosis on A, osteomalacia on B and adynamic bone disease on C
Paget's dz
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.
Paget's Dz
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.
fibrous dysplasia
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
solitary bone cyst
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.
aneurysmal bone cyst
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
osteoid osteoma
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
chondroblastoma
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.
osteosarcoma
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.
giant cell tumor
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%)
ewing sarcoma
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
multiple myeloma
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
metastatic carcinoma
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
articular hyaline cartilage
ankylosing spondylitis
CPPD
pigmented villonodular synovitis
nodular fasciitis
nodular fasciitis
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
fibrosarcoma
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
pleomorphic spindle cell sarcoma
liposarcoma
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
rhabdomyosarcoma
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.
synovial sarcoma
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.
Osteopetrosis
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
Osteogenesis imperfecta
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)
Osteonecrosis
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.
Osteomyelitis
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
Osteomyelitis
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
Acute osteomyelitis
PMN infiltrate, nonviable trabecular bone and marrow

Comps: septicemia, acute bacterial arthritis, pathologic fx, chronic osteomyelitis, SCC, amyloidosis.
Chronic osteomyelitis
lymphocytes and plasma cells, marrow fibrosis
Comps: septicemia, acute bacterial arthritis, pathologic fx, chronic osteomyelitis, SCC, amyloidosis.
Osteoporosis
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)
Primary HyperPTH
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!
Osteomalacia/Rickets
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
Paget's disease
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.
Tuberculosis (Pott's disease)
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
Fibrous dysplasia
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
Non-ossifying fibroma
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
Solitary bone cyst
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.
Aneurysmal bone cyst
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
Osteoid osteoma
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
Osteoid osteoma
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
Osteoblastoma
similar to osteoid osteoma but larger, without nocturnal pain relieved by aspirin
spine and long bones, 10-35yo, M=F.
tx: curettage
Enchondroma
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
Enchondroma
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
Chondroblastoma
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.
Osteosarcoma
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.
Chondrosarcoma
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
Chondrosarcoma
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
Ewing sarcoma
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
Giant cell tumor of bone
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%)
Multiple myeloma
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
metastatic tumors
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
soft tissue
Nodular fascitis
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
soft tissue
fibromatosis
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)
soft tissue
fibrosarcoma
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
soft tissue
Malignant fibrous histiocytoma
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.
soft tissue
lipoma
most common soft tissue mass (benign)
gross: yellow, soft and lobulated
histo: well-differentiated adipocytes, no atypia or fibroblasts
soft tissue
liposarcoma
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
soft tissue
leiomyoma
benign smooth muscle tumor, subcutaneous or vessel wall
gross: painful, firm yellow nodule
histo: bland spindle cells, low mitotic rate
soft tissue
leiomyosarcoma
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.
soft tissue
rhabdomyosarcoma
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.
soft tissue
rhabdomyosarcoma
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.
soft tissue
synovial sarcoma
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.