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Pathology II PCC > Chapter 20 > Flashcards

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
Q

primitive neuroectodermal tumor (PNET)

A 
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
Q

Giant Cell Tumor of bone

A

arthritis like pain
mets to lungs
thin shell of cortex remains
knee

27
Q

osteoarthritis

A 
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
Q

Rheumatoid Arthritis

A 
autoimmune
inflammatory
Anti-CCP antibodies
rheumatoid factor
Pannus
ulnar deviation 
Ankylosis present 
symmetric
MCP & PIP
bouchards nodes
females
10-40
29
Q

juvenile RA

A

immune-mediated
large joints
<16 years (MC 1-6)

30
Q

seronegative spondyloarthropathies

A

NOT +RF
+HLA-B27
P.E.A.R. (psoriatic arthritis, enteropathic arthritis, ankylosing spondylitis, reactive arthritis)

31
Q

Ankylosing spondylitis

A 
aka marie-strumpell disease
morning stiffness > 30 min
nocturnal LBP
\+HLA-B27
Axial skeleton:  gluteal pain referral
syndesmophytes: bamboo spine
32
Q

Gout

A

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
Q

pseudogout

A 
CPPD/CPDD
altered pyrophosphate metabolism
sporadic
pyrophosphate crystal deposition
knee, wrist, shoulder
>50 years
34
Q

Suppurative arthritis

A

bacterial arthritis
knee
N. gonorrhoeae = sexually active females, subacute course, polyarthicular

35
Q

lyme arthritis

A

borrelia burgdorferi

migratory joint pain

36
Q

ganglion

A

no communication with joint

wrist: joint capsule/tendon sheath

37
Q

synovial cyst

A

communicates with joint

baker cyst: popliteal

38
Q

tenosynovial giant cell tumor

A
  • 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
Q

soft tissue tumors

A

 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
Q

lipoma

A

MC soft tissue tumor of adults

41
Q

Liposarcoma

A

 Malignant soft tissue tumor
 May mets to LUNGS
 Mutation: degradation of p53
 Deep soft tissues: retroperitoneum, thigh

42
Q

Nodular Fasciitis

A

soft tissue tumor
 Reactive fibroblastic mass
 Non-neoplastic; self-limited
Volar/palm brachium

43
Q

Myositis Ossificans

A 
soft tissue tumor
	Reactive, metaplastic bone
	Proximal extremity muscles (MC) 
	Adolescent athletes = 2⁰ to trauma
	DDx: osteosarcoma
44
Q

Fibromatosis

A 
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
Q

fibrosarcoma

A

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
Q

Rhabdomyosarcoma

A

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
Q

Leiomyoma

A

benign smooth muscle tumor
 Found anywhere with smooth ms cells
 MC fibroids in Uterus,

48
Q

Leiomyosarcoma

A

malignant smooth muscle tumor
 ‘cigar-shaped nuclei’
 Females MC
 Found in deep, extremities, retroperitoneum

49
Q

synovial sarcoma

A

malignant smooth muscle tumor
knee
aggressive, invasive

50
Q

What disease are Klippel-Feil syndrome & Sprengel’s Deformity
Associated with?

A

Dysostosis

Pathology II PCC (7 decks)

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