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msk and ct - step > pathology - bone disorders/tumours > Flashcards

pathology - bone disorders/tumours Flashcards

1
Q

what goes wrong in achondroplasia

A

failure of longitudinal bone growth/endochondral ossificiation bc chondrocyte proliferation is inhibited

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2
Q

genetics of achondroplasia please

A

FGFR3 muation that results in constitutive actvation and inhibited chondrocyte proliferation

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3
Q

compare sponatenous vrs AD inheritance of achondroplaisa

A

spontaneous = > 85%

AD the others

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4
Q

what increases rate of mutations leading to achondroplasia

A

increased paternal age

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5
Q

describe the AD fo achondroplasia

A

AD with full penentrance; homozygosity is lethal

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6
Q

what is the most common cause of dwarfism

A

achondroplasia

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7
Q

what is primary osteoporosis

A

trabecular/spongy bone loses mass and interconnectiosn despite normal bone minearlization and lab vaullues

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8
Q

how to diagnosse primary osteoporosis

A

DEXA and T scor eof < - 2.5

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9
Q

T score of < 2.5

A

osteoporosis

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10
Q

wht can cause primary osteoporosis

A

long term esogenosu steroid use
anticonvulstand
anticoagulates
thyroid replacement theraphy

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11
Q 
what do these cause?
long term exogenous steroid use
anticonvulsants
anticoagulatneas
thyroid replacement therapy
A

primary osteoporosis

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12
Q

what are the most common types of fractures in osteoporisis

A

vertebral compression gractures
colles fracutres
fracutres of femoral neck

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13
Q

how do vetebral compression fractures present

A

acute back pain
loss of heighy
kyphosis

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14
Q

acute back pain
loss of height
kyphosis

A

primary osteoporisis with vertebral compression fracture

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15
Q

i say vertebral compression fractures, fracture at head fo femur and colle fracture you think?

A

primary osteoporosis

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16
Q

what causes type I primary osteoporosis?

A

post menopausal

increased bone resoprtion due to decreased estrogen

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17
Q

what is type II primary osteoporisis?

A

senile.

affects men and women > 70 years old

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18
Q

how to prophylaxis vrs primary osteoporisis type II senile

A

regular weight bearing exercise
adequte Ca and vitamin D thorughout adulthood
no smoking

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19
Q

treat primaroy osteoporosis

A

lifestyle modifications
bisphosphonase FIRST LINE
PTH analogs
SERMs (raloxifen/…? the one that isnet tamoxifen)
rarely calcitonin
DENOSUMAB (monoclonal RANKL, acts as osteoprotegrin)

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20
Q

inheritance pattern of osteopetrosis pelase

A

autosomal recessive

aka marble bone disease

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21
Q

what is osteopetrosis

A

failure of normal bone resportion due to defective osteoclasts

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22
Q

cxl indings in osteopetrosis please

A

thickeedn dense bones that are prone to fracture
bone fills marrow = pancytopenia and extramedullary hematopoiesis
BONE IN BONE at xray
cranial nerve impingement and palsies (hearing and vision loss) from narrowed foramina

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23
Q

xray of osteopetrosis

A

bone in bone

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24
Q

bone in bone

A

xray of osteopetrosis

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25
Q

genetics of osteopetrosis please

A

mutations in for ex carbonic anhydrase II impaired ability of required osteoblast generation of acidic environment necessary for bone reabsorption.
osteoblasts need alkaline environment.

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26
Q

what causes osteomalacia/rickets

A

vitamin D defieicny - defective mineralization/calcificaiton fo osteoid

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27
Q

defective minetalizaiton /caclification of osteoid

A

osteomalaica/rickets/vitamin D deficiency

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28
Q

soft, bowing out bones

A

osteomalacia/rickets (more so)

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29
Q

describe blood analysis in osteomalacia

A

decreased vitamin D - decreased ca and decreased PO4 - increased PTH
increased ALP - increased osteoblast hyperactivity

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30
Q

why is there increased ALP in osteomalacia?

A

osteoblast hyperactivity = increased ALP; osteoblasts require alkaline environment for functioning

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31
Q

osteitis defromans aka

A

paget disease of bone

32
Q

what causes page dz of bone

A

tis common

localized disorder of bone remodeling caused by increased in both osteoblastic and osteoclastic activity.

33
Q

describe blood analaysis of pagets

A 
normal ca
normal vit d
normal pho4
normal pth
increased ALP
34
Q

describe bone in pagets

A

mosaic patterh of woven and lamellar

35
Q

chal stick fractures in long bone = ?

A

paget disease

36
Q

complications in paget disease

A

high output cardiac failure = icnreased blood flow in bone due to icnreased arteriovenous shunts
increased risk of osteogenic sarcoma

37
Q

cxpx of pagets

A 
male > 50
at pelvis, skull, femur
BONE PAIN = most common complaint
head ache and hearing loss
increased hat size
38
Q

list the staged of paget disease pelase

A

lytic - osteoclasts mak lytic shaggy appearing lesions in bone
mixed - osteoclasts and osteoblasts
sclerotic - obsteoblasts = increased ALP
quiescent - minimal osteoclast/osteoblast activity

39
Q

xray finding on pagets

A

thickening of calvarium

40
Q

what am i?

h and e with osteocytes in lacuane and chatoic, mosaic pattern with lacy puple lines of bone remodelling

A

paget disease

41
Q

what is osteonecrosis

A

infarction of bone and marrow

avascular necorsis

42
Q

most common site of osteonecrosis/avascular necrosis

A

femoral head

due to insufficiency of medial circumflex artery

43
Q

what are teh causes of ostenoercosis

A

ASEPTCI
alcoholism, sickle cell dsiease, storage, exogenios and endocenous corticosteroids, pancreatitis ,trauma, legg calve perthes and caisson the bengs

44
Q 
what are these things:
caisson
endogenous and exogenous corticosteroids
sickle cell diseases
storage
alcoholsim
pancreatitis
trauma
legg calve perthes
A

causes of aseptic necrosis

45
Q

if you had to break your fever at its head, where would you prefer? pertrochangeric or supcapsular ?

A

peritrocahnteric - doesnt vut off the medial circumflex femoral artery - no aseptc necrosis

46
Q

lab values for OSTEOPOROSIS please

A 
normal ca
nomla po4
normal alp
normal pth
decreased bone mass
47
Q

lab vaules for OSTEOPETROSIS please

A 
normal ca or decreased
normal po4
normal alp
normal pth
dense, brittle bones.  calcium only decreased in severe disease or malignant disease
48
Q

labs for PAGET DIASES pelase

A 
normal calcium
normal PO4
increasd ALP
normal pth
abnomral mosaic bone architectures
49
Q

labs for OSTEOMALACIA/RICKETS

A 
decrease calcium
decreased PO4
increased ALP
increased PTH
soft bones
50
Q

labs for HYPERVITAMINOSIS

A 
increase calcium
increased PO4
no change in ALP
decreased PTH
granulomatous disease and oversupplementation cause it
51
Q

labs for primary hyperparathyroidism/osteitits dirbosa cystica

A 
increased ca
decreased po4
increased ALP
incrased PTH
brown tumors due to fibous replacemtn of bone, subperiosteal thinnking
52
Q

labs for secondary hyperparathyroidism/osteitis fibroisa cystica

A 
decreased ca
increased po4
increased ALP
increased PTH
ofetn due to ESRD
53
Q

list the primary benign bone tumours

A

giant cell

osteochondroma

54
Q

list teh primary maligi bone tumors

A

osteogenic sarcoma/oteosarcoma

ewing sarcoma

55
Q

age group for giant cell tumors

A

20-40 yo

56
Q

age group for osteochondroma

A

males < 25

57
Q

age group for osteogenic sarcoma/osteosarcoma

A

bimodal 10-20 and ? 65

58
Q

age group for ewing sarcoma

A

boys < 15 years

59
Q

location of giant cell tumor

A

epiphyseal end of long bones

60
Q

location of osteochondroma

A

exostosis at the metaphysis

61
Q

location of osteogenic sarcoma/osteosarcoma

A

metaphysis of long bones/near knee

62
Q

location fo ewign sarcoma

A

diaphysis of long pones, pelvis, scapula, ribs

63
Q

describe appaerance of giant cell tumor

A

multinuleared giant cells

soap bubble on xray (at epiphysis)

64
Q

describe appearbce of osteochnroma

A

exostosis with mature bone and cartilgiound cap

65
Q

describe apperance of osteogenic sarcoma/osteosarcoma

A

codman trianle - elevateion of periosteum or sunbust pattern on xray

66
Q

describe appearse of wewing sarcoma

A

anaplasti small blue cell maligi

onion skin periosteal reaction in bone

67
Q

t(11;22)

A

ewing sarcoma - EWS-FLI-1

68
Q

describe behaviour of giant cell

A

lolcaaly agressive bening around the knee

69
Q

describe behaviour of osteochondroma

A

rarely transforms to chondrosarcoma

70
Q

describe behaviour of osteogenic sarcoma/osteosarcoma

A

agressive - treat with suriflca en bloc resection with limb salvage and chemotheapy

71
Q

describe behaviour of ewing sarcoma

A

extermely aggressive with early mets

responsive to chemotherapy

72
Q

predipsosing factors for osteogenic sarcoma/osteosarcoma

A 
pagets
bone infarcts
radiation
familial retinoblastoma
li fraumeni
73
Q

list tumors found in diaphysis

A

round cell luesions - ewing sarcoma and myeloma
fibrous dysplasia
psteoid osteoma

74
Q

tumor with nighttime pain and central nidus

A

osteioid osteoma - diaphsys

75
Q

list the tumors in the metaphysis

A

osteosarcoma and osteochondroma and simple bone cyst

76
Q

list the tumros int eh epeiphysis

A

giant cell tumor

msk and ct - step (6 decks)

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