Module 13 Ch26 Bones, Joints, And Soft-Tissue Tumors

Front 1

receptors on osteoblasts bind what chemicals?

Back 1

hormones (PTH, vit D, leptin, estrogen)
cytokines
growth factors
extracellular matrix proteins

Front 2

what makes osteoblasts transform into osteocytes?

Back 2

if they are surrounded by newly deposited organic matrix

Front 3

osteoclasts are defived from progenitors that also give rise to what?

Back 3

monocytes and macrophages

Front 4

which cytokines and growth factors regulate osteoclast differentiation and maturation?

Back 4

M-CSF, IL-1, TNF

Front 5

what pathways regulate bone homeostasis?

Back 5

RANK/RANKL/OPG pathway

M-CSF/M-CSFr pathway

WNT/b-catenin/OPG pathway

Front 6

which two factors oppose each other in bone homeostasis?

Back 6

RANKL and OPG

Front 7

noncollagenous protein measured in the serum for osteoblast activity

Back 7

osteocalcin

Front 8

what factors determine age of peak bone mass?

Back 8

polymorphisms in receptors for vit D and LRP5/6, nutrition, physical activity, age, hormonal status

Front 9

which type of bone growth uses cartilaginous model?

Back 9

endochondral (longitudinal) ossification

Front 10

mutation of HOXD13 gene produces what abnormalities?

Back 10

extra digit between 3rd and 4th digit and syndactyly

Front 11

loss-of-fxn mutation of RUNX2 gene results in what abnormality?

Back 11

cleidocranial dysplasia (loss of osteoblastogenesis and chondrocyte activity); patent fontanelles, delayed closure of cranial sutures, Wormian bones, delayed tooth eruption, primitive clavicles, short height

Front 12

most common disease of the growth plate?

Back 12

achondroplasia

Front 13

pathogenesis of achondroplasia?

Back 13

mutated FGFR3 inhibits chondrocyte proliferation --> lack of longitudinal bone growth

Front 14

phenotype of pt affected by achondroplasia?

Back 14

short limbs with normal sized trunk and normal sized head w/ bulging forehead and depressed root of nose

Front 15

pathogenesis of thanatophoric dwarfism?

Back 15

gain-of-fxn mutation of FGFR3

Front 16

phenotype of thanatophoric dwarfism?

Back 16

short limbs, frontal bossing, relative macrocephaly, small chest cavity, bell-shaped abdomen

Front 17

Endosteal hypreostosis, Van Buchem dz, and autosomal dominant osteopetrosis 1 are caused by what mutation?

Back 17

gain-of-fxn mutation of LPR5
(cell surface receptor that activates WNT/B-catenin pathway in osteoblasts)

Front 18

osteoporosis pseudoglioma syndrome is caused by what mutation?

Back 18

loss-of-fxn mutation of LPR5 (cell surface receptor that activates WNT/B-catenin pathway in osteoblasts)

Front 19

most commonly inherited disorder of CT?

Back 19

osteogenesis imperfecta (brittle bone disease)

Front 20

what determines whether phenotype of osteogenesis imperfecta will be mild or severe?

Back 20

mild = decreased synthesis of normal collagen
severe = abnml polypeptide chains that can't be assembled into collagen

Front 21

disease that results from autosomal dominant mutations of genes for α1 and α2 chains of collagen?

Back 21

osteogenesis imperfecta

Front 22

disease that is fatal in utero and has extraordinary bone frailty with multiple intrauterine fracutres?

Back 22

osteogenesis imperfecta type II

Front 23

pts with this disease have normal life span w/ childhood fractures that decrease at onset of puberty; blue sclera; hearing loss; dental imperfections

Back 23

osteogenesis imperfecta type I

Front 24

what is type 1 collagen disease called?

Back 24

osteogenesis imperfecta

Front 25

diseases associated with mutations of types 2, 9, 10 and 11 collagen have defects in what type of tissue?

Back 25

hyaline cartilage

Front 26

deficiencies in enzymes that degrade dermatan sulfate, haparan sulfate and karatan sulfate are called what?

Back 26

mucopolysaccharidoses

Front 27

mucopolysaccharidoses cause problems in what process?

Back 27

cartilage formation
(chondrocytes metabolize ECM mucopolysaccharides)

Front 28

mucopolysaccharidoses manifest in what structures?

Back 28

cartilage anlage, growth plates, costal cartilages, articular surfaces

Front 29

basic problem in osteopetrosis?

Back 29

mutations interfere with acidification of osteoclast resorption pit

Front 30

mutations of which genes cause the various forms of osteopetrosis?

Back 30

CA2
CLCN7
TCIRG1
RANKL

Front 31

morphologic chgs of bones in osteopetrosis?

Back 31

bones lack medullary canal, ends of long bones are bulbous and misshapen, primary spongiosa is not removed, bones are brittle

Front 32

bone abnormality presenting with fracture, anemia, hydrocephaly, CN defects, repeated infections?

Back 32

osteopetrosis

Front 33

when the general term "osteoporosis" is used, what forms is it referring to?

Back 33

senile and postmenopausal osteoporosis

Front 34

what is an example of low-turnover variant osteoporosis?

Back 34

senile osteoporosis

Front 35

what is an example of high-turnover variant osteoporosis?

Back 35

postmenopausal osteoporosis

Front 36

pathogenesis of postmenopausal osteoporosis?

Back 36

dec estrogen --> inc inflammatory cytokines from monocytes and BM cells --> inc osteoclast recruitment and activity

Front 37

postmenopausal osteoporosis most frequently affects what areas?

Back 37

cancellous compartment of vertebral bodies

Front 38

mosaic bone pattern, inc Alk Phos, possible high-output heart failure. Dx?

Back 38

Paget's disease (osteitis deformans)

Front 39

severe complication of Paget's disease?

Back 39

development of osteosarcoma

Front 40

in children, it's called rickets; in adults, it's called osteomalacia - disorder?

Back 40

vitamind D deficiency or disturbance in its metabolism

Front 41

abnml lab values in osteomalacia?

Back 41

dec Ca, inc PTH, dec serum phosphate

Front 42

increased bone cell activity, peritrabecular fibrosis, cystic brown tumors?

Back 42

osteitis fibrosa cystica, hyperparathyroidism

Front 43

what causes brown tumors?

Back 43

osteitis fibrosa cystica

microfractures and secondary hemorrhage elicit influx of macrophages and ingrowth of fibrous tissue

Front 44

manifestations of chronic renal dz in bone?

Back 44

- inc bone resorption
- osteomalacia
- osteosclerosis
- growth retardation
- osteoporosis

Front 45

pathogenesis of bone changes in renal failure?

Back 45

- phosphate retention --> inc PTH
- dec conversion of Vit D --> dec reabs of Ca and inc PTH
- metabolic acidosis --> inc bone resorption

Front 46

MS complication of hemodialysis?

Back 46

deposition of amyloid in bone and periarticular structures

Front 47

closed (simple) fracture?

Back 47

skin is intact

Front 48

compound fracture?

Back 48

fracture communicates with skin surface

Front 49

comminuted fracture?

Back 49

bone is splintered

Front 50

most common regions of osteonecrosis?

Back 50

medullary cavity of metaphysis or diaphysis, subchondral region of epiphysis

Front 51

which type of osteonecrosis is worse: subchondral or medullary?

Back 51

subchondral, as it is associated with chronic pain and collapse, predisposing to severe secondary osteoarthritis

Front 52

in adults, osteomyelitis occurs as a complication of what?

Back 52

open fractures, surgical procedures, diabetic infections of the feet

Front 53

in children, osteomyelitis occurs as a complication of what?

Back 53

trivial injuries to mucosa or minor skin infxns

Front 54

MCC of pyogenic osteomyelitis?

Back 54

S. aureus

Front 55

MCCs of pyogenic osteomyelitis in pts w/ GU infxn or IVDU?

Back 55

E. coli, Pseudomonas, Klebsiella

Front 56

most common site of osteomyelitis in neonate?

Back 56

metaphysis, epiphysis or both

Front 57

most common site of osteomyelitis in children?

Back 57

metaphysis

Front 58

most common site of osteomyelitis in adults?

Back 58

epiphyses and subchondral regions

Front 59

how is an involucrum formed?

Back 59

chronic inflammatory cells around initial infxn release cytokines that stimulate bone resorption and peripheral bone deposition

Front 60

small intraosseous abscess of cortex, walled off by reactive bone?

Back 60

Brodie abscess

Front 61

complication of osteomyelitis that develops in the jaw, assocd with extensive new bone formation that obscures underlying bone structure?

Back 61

sclerosing osteomyelitis of Garre

Front 62

on x-ray of focal bone pain, you see a lytic lesion surrounded by a zone of sclerosis. Dx?

Back 62

osteomyelitis

Front 63

what can lead to chronicity in osteomyelitis?

Back 63

delay in dx, extensive bone necrosis, inadequate abx therapy or debridement, weakened host defenses

Front 64

difference btwn pyogenic and tuberculous osteomyelitis?

Back 64

tuberculous is more destructive and resistant to control and has tropism for the spine, hips and knees

Front 65

pt presents with low grade fever, chills, night sweats, wt loss, low back tenderness w/ pain on motion. Dx?

Back 65

Tuberculous osteomyelitis

Front 66

when do bone lesions begin to appear in congenital syphilis?

Back 66

5th month of gestation

Front 67

in acquired syphilis, when does bone disease begin?

Back 67

early tertiary phase (2-5 y after infxn)

Front 68

cause of saber shin?

Back 68

skeletal syphilis
(massive reactive periosteal bone deposition on medial and anterior tibial surfaces)

Front 69

most common primary cancer of bone?

Back 69

osteosarcoma

Front 70

most common benign bone tumors?

Back 70

osteochondroma and fibrous cortical defect

Front 71

bosselated, round-to-oval sessile tumors of subperiosteal surface of cortex, on or inside skull and facial bones; usually solitary, detected in middle age

Back 71

osteoma

Front 72

benign tumor <2cm in pt <25; often involve posterior spine, femur, or tibia; cause severe nocturnal pain responsive to aspirin

Back 72

osteoid osteoma

Front 73

benign tumor >2cm; usually in the spine; dull, achy pain unresponsive to aspirin; does not induce bony reaction

Back 73

osteoblastoma

Front 74

benign tumor associated with Gardner's syndrome (FAP), grows on existing bone?

Back 74

osteoma

Front 75

benign tumors of interlacing trabeculae of woven bone surrounded by osteoblasts?

Back 75

osteoid osteoma and osteoblastoma

Front 76

malignant tumor most common in men age 10-20 in metaphysis of long bones (esp around knee); elevation of periosteum produces "Codman's triangle" on x-ray

Back 76

Osteosarcoma

Front 77

most common benign bone tumor, usually in men < 25 on long metaphysis, cartilage-capped tumor attached by bony stalk,

Back 77

osteochondroma (exostosis)

Front 78

benign cartilaginous tumor usually found in intramedullary bone, usually of short tubular bones of hands and feet

Back 78

chondroma/enchondroma

Front 79

rare, benign cartilaginous tumor most commonly around the knee in young males; chicken-wire pattern of mineralization?

Back 79

chondroblastoma

Front 80

rarest cartilage tumor, variable morphology, most often young males, in metaphysis of long tubular bones; on x-ray, eccentric lucency, well delineated from adjacent bone by rim of sclerosis

Back 80

chondromyxoid fibroma

Front 81

malignant cartilaginous tumor of men age 30-60, usually in pelvis, spine, scapula, humerus, tibia, or femur; expansive glistening mass within medullary cavity

Back 81

chondrosarcoma

Front 82

developmental defect found in 30-50% of children >2 y.o., most arise eccentrically in metaphysis of distal femur and prox tibia

Back 82

fibrous cortical defects
(non-ossifying fibroma if grows to >5-6cm)

Front 83

benign tumor in which bone replaced by fibroblasts, collagen and irreg bony trabeculae, affects many bones; essentially a localized developmental arrest

Back 83

fibrous dysplasia

Front 84

multiple unilateral bone lesions assocd with endocrine abnormalities (precocious puberty) and unilateral pigmented skin lesions (cafe-au-lait spots)?

Back 84

McCune-Albright syndrome
(polyostotic fibrous dysplasia)

Front 85

anaplastic small blue cell malignant tumor most common in boys < 15; assoc'd with t(11;22); onion-skin appearance in bone; commonly in diaphysis of long bones, pelvis, scapula, and ribs

Back 85

Ewing's sarcoma

(primitive neurectodermal tumor is a variant)

Front 86

benign but locally aggressive tumor at epiphyseal end of long bones esp around knee; peak age 20-40; "soap bubble" on x-ray; spindle-shaped cells w/ multinucleated giant cells

Back 86

giant cell tumor (osteoclastoma)

Front 87

benign tumor w/ blood-filled spaces; most often in pts <20; most often in metaphyses of long bones and posterior vertebral bodies

Back 87

aneurysmal bone cyst

Front 88

most common form of skeletal malignancy?

Back 88

metastatic tumors

Front 89

most common origins of metastasis to bone in adults?

Back 89

cancers of prostate, breast, kidney, and lung

Front 90

most common origins of metastasis to bone in children?

Back 90

Wilms tumor, osteosarcoma, neuroblastoma, Ewing sarcoma, rhabdomyosarcoma

Front 91

cause of lytic bone lesions in metastatic tumors?

Back 91

metastatic cells secrete PGs, cytokines, and PTH-related protein that stimulate osteoclast activity

Front 92

why do metastatic tumors grow well in bone?

Back 92

lysis of bone tissue rich in TGF-β, IGF-1, FGF, PDGF, and bone morphogenetic proteins helps create environment conducive to tumor cell growth

Front 93

what activates degradative enzymes that break down cartilage?

Back 93

IL-1 and TNF from chondrocytes, synoviocytes, fibroblasts, and inflammatory cells

Front 94

the pathology of this disease of joints is d/t intrinsic breakdown of cartilage, not inflammation

Back 94

osteoarthritis

Front 95

causes of secondary osteoarthritis?

Back 95

previous injuries to a joint, congenital joint deformity, diabetes, ochronosis, hemochromatosis, marked obesity

Front 96

complications of osteoarthritis?

Back 96

subchondral cysts, sclerosis, osteophytes, eburnation, heberden's nodes

Front 97

deep, achy pain that worsens with use, morning stiffness, crepitus and limitation of ROM?

Back 97

osteoarthritis

Front 98

what joints are typically spared in osteoarthritis?

Back 98

wrists, elbows, shoulders

Front 99

chronic, inflammatory disorder producing nonsuppurative proliferative and inflammatory synovitis that often progresses to destruction of the cartilage and ankylosis of the joints?

Back 99

rheumatoid arthritis

Front 100

RA is seen ____ frequently in women than in men

Back 100

more (3-5x)

Front 101

RA has strong association with what HLA antigen?

Back 101

HLA-DR4

Front 102

what causes the joints to be red and warm in RA?

Back 102

increased vascularity d/t vasodilation and angiogenesis

Front 103

cause of subchondral cysts and osteoporosis in RA?

Back 103

osteoclastic activity in underlying bone allows synovium to penetrate into bone

Front 104

where is pannus formation most often seen?

Back 104

MCP, PIP

Front 105

what joints are characteristically spared in RA?

Back 105

DIP, lumbosacral spine, hips

Front 106

what are rheumatoid nodules?

Back 106

fibrinoid necrosis surrounded by palisading histiocytes; arise in skin regions subject to pressure (ulnar FA, elbows, occiput, lumbosacral area)

Front 107

40 yo female w/ BL pain, warmth, and swelling in ankles, fingers, and feet with morning stiffness?

Back 107

Rheumatoid arthritis

Front 108

purpura, cutaneous ulcers and nail bed infarction d/t leukocytocastic venulitis is seen in what disease?

Back 108

RA

Front 109

pathogenesis of RA?

Back 109

activation of CD4+ --> stimulate synoviocytes and macrophages --> release proinflammatory mediators (IL-1, IL-6, IL-23, TNF, PGE2, NO, TGF-b, GM-CSF) --> activates endothelial cells --> increased leukocyte binding --> Ag-Ab complexes destroy cartilage and stimulate osteoclasts

Front 110

radiographic hallmarks of RA?

Back 110

joint effusions, juxta-articular osteopenia w/ erosions and narrowing of joint space w/ loss of articular cartilage

Front 111

joint involvement pattern in RA?

Back 111

usually symmetrical, small joints before larger ones (hands, feet first)

Front 112

therapies for RA?

Back 112

corticosteroids, methotrexate, antagonists of TNF

Front 113

how does JIA differ from RA in adults?

Back 113

- oligoarthritis is more common
- systemic dz more frequent
- large joints > small joints
- no rheumatoid nodules or RF
- ANA + is common

Front 114

remitting, high spiking fevers, migratory and transient skin rash, hepatosplenomegaly, and serositis?

Back 114

Systemic arthritis-type JIA

Front 115

what type of hypersensitivity reaction is RA?

Back 115

type III

Front 116

what is rheumatoid factor?

Back 116

anti-IgG antibody, present in 80% of RA pts

Front 117

antibody other than RF used in dx of RA?

Back 117

anti-CCP Ab

Front 118

what systemic sxs are assocd with RA?

Back 118

fever, fatigue, pleuritis, pericarditis

Front 119

seronegative spondyloarthropathies have strong association with what?

Back 119

HLA-B27

Front 120

chronic inflammatory dz of spine and sacroiliac joints --> stiff spine, uveitis, and AR; RF negative.

Back 120

Ankylosing spondylitis

Front 121

triad of conjuctivitis/anterior uveitis, urethritis, and arthritis; caused by autoimmune rxn initiated by GI or chlamydial infxn

Back 121

Reiter syndrome (reactive arthritis)

Front 122

pt presents with thick red patches of skin with silvery-white scales, asymmetric dactylitis of DIPs and swelling of ankles, knees, hips, or wrists?

Back 122

psoriatic arthritis

Front 123

MCC acute suppurative arthritis?

Back 123

bacterial arthritis

Front 124

MCC bacterial arthritis in children under 2?

Back 124

H. influenzae

Front 125

MCC bacterial arthritis in older children and adults?

Back 125

S. aureus

Front 126

gonococcal arthritis is prevalent in what age groups?

Back 126

late adolescents and young adults

Front 127

Salmonella is a cause of bacterial arthritis in what subset of pts?

Back 127

sickle cell disease pts

Front 128

sudden development of single acutely painful and swollen joint w/ restricted ROM; fever, leukocytosis, elevated ESR?

Back 128

bacterial arthritis

Front 129

most common joint affected by bacterial arthritis?

Back 129

knee

Front 130

chronic, progressive monoarticular disease w/ confluent granulomas w/ central caseous necrosis most commonly in hips, knees, or ankles?

Back 130

tuberculous arthritis

Front 131

60-80% of untreated cases of what disease develop arthritic sxs in large joints?

Back 131

Lyme disease

Front 132

monoarticular, migratory arthritis with asymmetrical pattern; presents with synovitis, tenosynovitis, and dermatitis?

Back 132

gonococcal arthritis

Front 133

most individuals with chronic gout also develop what?

Back 133

urate nephropathy

Front 134

uric acid is the end product of what?

Back 134

purine metabolism

Front 135

hyperuricemia develops from what in 90% of cases?

Back 135

reduced excretion of uric acid

(other 10% is from overproduction)

Front 136

deficiency of what enzyme leads to increased purine synthesis by the de novo pathway?

in what inherited disorder is this seen?

Back 136

HGPRT

Lesch-Nyhan syndrome (x linked)

Front 137

what factors contribute to development of primary gout?

Back 137

- Age
- inheritance
- alcohol consumption
- obesity
- thiazides
- Pb toxicity

Front 138

what attracts neutrophils to sites of MSU crystal deposition?

Back 138

MSU phagocytosed by macrophages => IL-1b, IL-18 => inc adhesion molecules and neutrophil chemokine CXCL8

Front 139

what produces the actual tissue injury and inflammation of acute gouty arthritis?

Back 139

neutrophils that phagocytose crystals release PGs and free radicals, and are eventually lysed and release lysosomal enzymes into joint

Front 140

what are tophi?

Back 140

large aggregates of precipitated crystals and the surrounding inflammatory reaction

Front 141

first attack of gout usually occurs where?

Back 141

1st MTP

Front 142

systemic diseases associated with gout?

Back 142

CV disease (atherosclerosis and HTN)

renal colic --> nephropathy

Front 143

asymmetric joint disease w/ nodule formation on external ear, olecranon, or Achilles tendon; acute episodes occur after large meal or alcohol consumption?

Back 143

gout

Front 144

crystals aspirated in joint space are weakly birefringent, basophilic, rhomboid shape

Back 144

pseudogout

Front 145

crystal deposition disease more common in men

Back 145

gout

Front 146

why does alcohol predispose to gouty attack?

Back 146

it competes for same excretion sites in kidney as uric acid, causing dec uric acid secretion and buildup in blood

Front 147

small cyst almost always located near joint capsule or tendon sheath?

Back 147

ganglion cyst

Front 148

herniation of synovium through a joint capsule or massive enlargement of a bursa may produce what?

Back 148

a synovial cyst

Front 149

benign neoplasms of synovial linings, tendon sheaths and bursae; heavily infiltrated by macrophages that may have hemosiderin and lipid filled vacuoles; may form multinucleated giant cells?

Back 149

tenosynovial giant-cell tumor

Front 150

solitary, slow-growing, painless mass often on tendon sheaths of wrists and fingers, most common mesenchymal neoplasm of the hand?

Back 150

localized tenosynovial giant-cell tumor

Front 151

soft, well-encapsulated fat tumor. Benign. Simple excision is usually curative.

Back 151

Lipoma

Front 152

malignant fat tumor that can be large. Will recur unless adequately excised.

Back 152

liposarcoma

Front 153

Benign tumor derived from striated muscle. May occur in heart in tuberous sclerosis.

Back 153

rhabdomyoma

Front 154

Most common soft tissue tumor of childhood. malignant. Arises from skeletal muscle, most often in head/neck.

Back 154

Rhabdomyosarcoma

Front 155

mimics sarcoma, occurs in adults on volar aspect of forearm, also on chest and back. Deep dermis, subcutis, or muscle

Back 155

nodular fasciitis

Front 156

bone metaplasia that arises in musculature of athletic young adults, often after trauma. Initially filled with fibroblast/myofibroblast-like cells, eventually becoming ossified

Back 156

myositis ossificans

Front 157

similar malignant disease to myositis ossificans?

what is the major clinical difference?

Back 157

extraskeletal osteosarcoma

osteosarcoma occurs in elderly and lacks zonation of myositis ossificans

Front 158

benign nodular or broad fascicles of fibroblasts and myofibroblasts surrounded by abundant dense collagen, leading to contractures of the 4th and 5th digits of the hand and abnml curvature of the penis and urethral constriction

Back 158

Superficial fibromatosis

Front 159

what is the palmar variant fibromatosis called?

Back 159

Dupuytren contracture

Front 160

what is the penile variant fibromatosis called?

Back 160

Peyronie disease

Front 161

large, infiltrative masses of well-differentiated fibroblasts that do not metastasize, but may recur after excision; have mutations in APC or b-catenin gene

Back 161

deep-seated fibromatosis

Front 162

arises in musculoaponeurotic structures of anterior abd wall in women during or after pregnancy

Back 162

abdominal fibromatosis

Front 163

aggressive, malignant neoplasm commonly occuring in deep soft tissues of extremities. Unencapsulated, infiltrative, soft, fish-flesh masses w/ areas of hemorrhage and necrosis

Back 163

Fibrosarcoma

Front 164

benign fibroblast neoplasm f the dermis and subcutis. Painless, slow-growing, firm, small, mobile nodule of middle aged adults.

Back 164

Benign fibrous histiocytoma (dermatofibroma)

Front 165

skeletal muscle neoplasms are almost all ______?

Back 165

malignant

Front 166

benign smooth muscle tumors often arising in the uterus

Back 166

leiomyomas

Front 167

hereditary leiomyomatosis has a predisposition to develop what other neoplasm?

Back 167

renal cell carcinoma

Front 168

malignant tumor of smooth muscle that affects adults, women > men, mostly in skin and deep soft tissues of extremities and retroperitoneum

Back 168

leiomyosarcoma

Front 169

malignant neoplasm of unclear origin, pt age 20-40, arises in deep soft tissue, esp around knee/thigh; commonly biphasic or monophasic on histology. Positive for keratin and epithelial membrane antigen

Back 169

Synovial sarcoma