Chapter 20 Flashcards

1
Q

Dysostosis

A
congenital
effects bone
•Abnormal mesenchymal migration
•	Aplasia
•	Supernumerary digits
•	Abnormal fusion of bones  Klippel-Feil syndrome & Sprengel’s Deformity
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2
Q

Dysplasia

A
congenital
bone & cartilage
•	Not pre-cancerous
•	In bone: osteodysplasia
•	In cartilage: chondrodysplasia
•	Dwarfism
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3
Q

Osteogenesis Imperfecta (O.I.)

A
congenital
collagen
•	Aka brittle bone disease
•	Abnormal collagen: premature breakdown
•	Mutations of type I collagen: Autosomal dominant
•	ECM
o	Blue sclera
o	Type I: normal lifespan
o	Type II: lethal in utero
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4
Q

Achondroplasia

A
congenital
cartilage
•	↓ cartilage synthesis = ↓ growth plate expansion 
•	Spontaneous: 75%
•	Autosomal Dominant: 25%
•	MC form of dwarfism
•	Thanatophoric dwarfism = fatal
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5
Q

osteopetrosis

A
congenital
bone
•	↓ osteoclast-mediated bone resorption
•	Skeletal sclerosis—↑ fractures (chalk stick)
•	Foraminal stenosis CN palsies
•	Medullary cavity fills
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6
Q

osteoporosis

A
bone
females & ↑ age
•	Most important form of osteopenia
•	Osteoclast/osteoblast imbalance
•	MC impacts spongy bone
•	1⁰ Senile, PM (MC)
1.	Vertebral compression fx 
2.	Femoral neck fx 
	Xrays DONT diagnose! 
TX: Estrogen therapy
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7
Q

Paget disease (osteitis deformans)

A
Bone
70 years old
Males
	Phases: lytic, mixed, sclerotic
	‘Shaggy’ xray & ivory vertebra sign
	Regional osteoclastic activity
	Excessive bone formation 
MC asymptomatic
	Bone pain (neck & back) MC
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8
Q

Rickets & osteomalacia

A

Bone
kids = rickets = more severe
adults = osteomalacia
 Hypocalcemia –>PTH –> demineralizes bone

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9
Q

Hyperparathyroidism

A
Bone
PM women
	MC nonmalignant cause of hypercalcemia
	Cortical & trabecular bone
MC because of adenoma
‘Brown tumor’
Renal stones MC
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10
Q

Avascular Necrosis/ osteonecrosis

A
Bone
subchondral areas
preserve cortex
osteochondritis dissecans
MC vascular disruption via fx
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11
Q

Osteomyelitis

A

Bone marrow inflammation
Acute
Categories: pyogenic bacteria, tuberculosis or mixed
Hematogenous spread

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12
Q

Pyogenic

Osteomyelitis

A

Bone
Pyogenic bacteria
Overall MC- S. aureus

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13
Q

Tuberculous osteomyelitis

A

Bone
Skeletal TB
Caseous granulomas
Hematogenous spread MC

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14
Q

Bone Tumors (general)

A

 Matrix & fibrous producing tumors MC
 Benign tumors MC
 MC develop early (50% at knee
 Osteoma: age 40-50, facial bones/skull

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15
Q

osteoma

A

benign
bone forming tumor
found on head
middle age

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16
Q

osteoid osteoma

A
benign bone forming tumor
nocturnal pain
aspirin relieves
prox femur
Male
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17
Q

osteoblastoma

A

benign bone forming tumor
unrelieved by aspirin
vertebral

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18
Q

osteosarcoma

A
Malignant bone forming CA
aggressive
MC primary bone CA
	Periosteal & medullary destruction (Codman triangle, sunburst, spiculations) 
Knee
Male
young/typical/primary = 10-20 years, long term survival
increased risk for retinoblastoma
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19
Q

osteochondroma/exostosis

A
benign cartilage forming tumor
exostoses = cartilage-capped outgrowths
increased risk for sarcoma
knee
males
puberty
solitary-- adolescence/young adults
multiple--genetic, childhood
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20
Q

chondroma/enchondroma

A
benign cartilage forming tumor
solitary or multiple lesions
'o-ring' sign
MC asymptomatic
Ollier disease: multiple lesions, unilateral
tubular bones of hand & feet
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21
Q

chondrosarcoma

A
malignant cartilage forming tumor
less common than osteosarcomas
thicken or erode cortex
low-grade= MC, thickens cortex, good prognosis
high-grade= cortical erosion, mets to lungs, poor prognosis, appears dark on film
intramedullary MC
large prox joints
older males
22
Q

Fibrous Cortical Defect

A
Benign fibrous tumor
NOT A TRUE NEOPLASM
small
asymptomatic
knee (distal femur)
30-50% of all kids >2
Nonossifying fibroma = larger fibrous cortical defect
23
Q

fibrous dysplasia

A

benign
arrested development
monostotic MC
polyostotic = no endocrine, severe deformation, crainofacial involvement
McCune-Albright = coast of Maine, precocious puberty,females

24
Q

Ewing sarcoma

A
second MC pediatric bone cancer
like PNET
undifferentiated
femur
10-20
Caucasian males
periosteum: onion-skinning, sunburst
25
primitive neuroectodermal tumor (PNET)
``` bone cancer neural differentiation like Ewing Sarcoma homer-wright rosettes similar to medulloblastoma but not in CNS periosteum: onion-skinning, sunburst femur 10-20 Caucasian males ```
26
Giant Cell Tumor of bone
arthritis like pain mets to lungs thin shell of cortex remains knee
27
osteoarthritis
``` MC joint disorder degeneration of articular cartilage subchondral changes are secondary mechanical and genetic causes not always inflammation present worse in morning for a short time Heberden's node at DIP joint NO ankylosis Females: knee & hands males: hips traumatic/deformity --> can occur in kids ```
28
Rheumatoid Arthritis
``` autoimmune inflammatory Anti-CCP antibodies rheumatoid factor Pannus ulnar deviation Ankylosis present symmetric MCP & PIP bouchards nodes females 10-40 ```
29
juvenile RA
immune-mediated large joints <16 years (MC 1-6)
30
seronegative spondyloarthropathies
NOT +RF +HLA-B27 P.E.A.R. (psoriatic arthritis, enteropathic arthritis, ankylosing spondylitis, reactive arthritis)
31
Ankylosing spondylitis
``` aka marie-strumpell disease morning stiffness > 30 min nocturnal LBP +HLA-B27 Axial skeleton: gluteal pain referral syndesmophytes: bamboo spine ```
32
Gout
abnormal purine metabolism -- inc uric acid Tophus/Tophi primary = hyperuricemia secondary = co-morbid or drug exposure chronic tophaceous arthritis = doesnt resolve, can die of gouty nephropathy MC hallux = podagra males
33
pseudogout
``` CPPD/CPDD altered pyrophosphate metabolism sporadic pyrophosphate crystal deposition knee, wrist, shoulder >50 years ```
34
Suppurative arthritis
bacterial arthritis knee N. gonorrhoeae = sexually active females, subacute course, polyarthicular
35
lyme arthritis
borrelia burgdorferi | migratory joint pain
36
ganglion
no communication with joint | wrist: joint capsule/tendon sheath
37
synovial cyst
communicates with joint | baker cyst: popliteal
38
tenosynovial giant cell tumor
- genetic mutation promotes growth - diffuse: lg joints=knee, PAIN, finger-like projections - localized: small joints=hand, MC soft tissue tumor of hand, PAINLESS - 20-50 years
39
soft tissue tumors
 ANY non-epithelial tissue except: bone, cartilage, CNS, hematopoietic or lymphoid tissue INCLUDES PNS  Benign 100x MC than malignant  EXCEPT SKELETAL MS  Soft tissue sarcomas disproportionally aggressive (<1% Dx but 2% of ca-related death)  MC in lower extremities (thigh)
40
lipoma
MC soft tissue tumor of adults
41
Liposarcoma
 Malignant soft tissue tumor  May mets to LUNGS  Mutation: degradation of p53  Deep soft tissues: retroperitoneum, thigh
42
Nodular Fasciitis
soft tissue tumor  Reactive fibroblastic mass  Non-neoplastic; self-limited Volar/palm brachium
43
Myositis Ossificans
``` soft tissue tumor  Reactive, metaplastic bone  Proximal extremity muscles (MC)  Adolescent athletes = 2⁰ to trauma  DDx: osteosarcoma ```
44
Fibromatosis
``` benign soft tissue tumor  Disfiguring, painful, commonly recur 1. Superficial: superficial fascia, deformation Dupuytren contracture, Peyronie disease (MC 40-70 yrs) 2. Deep/desmoid tumors: MC abdominal Aggressive, locally destructive, Gardner syndrome: genetic mutation, multiple deep fibromatoses, intestinal polyposis, osteomas ```
45
fibrosarcoma
malignant soft tissue tumor herringbone pattern of cells 25% mets to lungs 50% recur after excision deep tissues: thigh, retroperitoneum, knee originates in soft tissue then invades bone
46
Rhabdomyosarcoma
malignant skeletal muscle tumor  Rhabdomyoblasts: skeletal muscle precursor  MC pediatric soft tissue sarcoma  Occurs in areas w minimal skeletal ms: Head/neck (MC),  2/3 kids cured  Adults: poor prognosis
47
Leiomyoma
benign smooth muscle tumor  Found anywhere with smooth ms cells  MC fibroids in Uterus,
48
Leiomyosarcoma
malignant smooth muscle tumor  ‘cigar-shaped nuclei’  Females MC  Found in deep, extremities, retroperitoneum
49
synovial sarcoma
malignant smooth muscle tumor knee aggressive, invasive
50
What disease are Klippel-Feil syndrome & Sprengel’s Deformity Associated with?
Dysostosis