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Year2 MSK exam 1 > MSK path IV > Flashcards

MSK path IV Flashcards

1
Q

tumor with schwann cell differentiation

A

peripheral nerve sheath tumor

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2
Q

3 common types of peripheral nerve sheath tumor

A

schwannoma
neurofibroma
malignant peripheral nerve sheath tumor

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3
Q

peripheral nerve sheath tumors are assoc with what familial tumor syndromes

A

NF I, NF II, schwannomatosis

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4
Q

what is a consistent finding in all schwannomas

A

loss of expresion of NF 2 that produces merlin

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5
Q

schwannomas

A

benign tumors that exhibit schwann cell differentiation and arise from peripheral nn

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6
Q

Antoni A and Antoni B

A

A- cellular
B- noncellular
schwannoma

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7
Q

Verocary bodies

A

anuclear rows

schwannoma

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8
Q

Sx from schwannomas

A

usually from compression

tinnitus, hearing loss

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9
Q

in cranial vault where are schwannomas

A

cerebellopontine angle where attach to CN VIII

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10
Q

acoustic neuroma

A

schwannoma in CPA

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11
Q

What is a neurofibroma

A

benign nerve sheath tumor
heterogenous in composition–neoplastic schwann cells admixed with perineurial like cells, fibroblasts, mast cells and CD34+ spindle cells

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12
Q

cell marker for neurofibroma

A

CD34+

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13
Q

pedunculated nodule with CD34+

A

supergicial cutaneous NF

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14
Q

pedunculated nodules all over with CD34+

A

NF1- associated

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15
Q

large plaquelike elevation of skin CD34+

A

diffuse NF, assoc NF1

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16
Q

plexiform neurofibromas

A

deep or superficial locations in association with nerve roots or large nerves and ALWAYS NF1 related

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17
Q

guaranteed to see what with NF-1

A

plexiform neurofibromas

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18
Q

what are malignant peripheral nerve sheath tumors though to arise from

A

malignant transformation of plexiform neurofibromas

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19
Q

divergent differentiation

A

focal areas that have other lines of differentiation

glandular, cartilaginous, osseous, rhabdomyoblastic morphology

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20
Q

triton tumor

A

Malignant PNST with divergent differentiation

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21
Q

cafe au lait spots

A

NF 1

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22
Q

NF 2

A

auto dominant
range of tumors
b/l CN VIII schwannoma and multiple meningiomas

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23
Q

composition of bone matrix

A

osteoid(35%) and mineral component(65%)

24
Q

what collagen is in bone

A

type I

25
Q

what regulates synthesis of boney matrix

A

hormonal and local mediators

26
Q

what indicates inactive osteoblasts

A

decrease in cytoplasm

27
Q

what are osteocytes

A

inactive osteoblasts that become embedded in matrix

28
Q

osteocytes help what

A

control Ca and phosphate levels in microenvironment and detect mechanical forces “mechanotransduction”

29
Q

specialized mutlinucleated macrophages in bone

A

osteoclasts

30
Q

how do osteoclasts break down bown

A

cell surface integrins form seal for resorption pit and then secretion of acid and neutral proteases (mmp) cause dissolution inorganic and organic components of bone

31
Q

how many nuclei are in osteoclasts

A

3-25

32
Q

osteoclast can be mis identified as what

A

megakaryocyte

33
Q

zones in active bone growth plate

A 
reserve zone
zone of proliferation
zone of hypertrophy
zone of mineralization
primary spongiosa
34
Q

how much of adult skeleton is replaced anually

A

10%

35
Q

peak bone mass

A

after cessation of skeletal growth

36
Q

role of OPG

A

bind up RANKL so osteoclasts cannot be activated

37
Q

what stimulates production RANKL

A

M-CSF on osteoblast

38
Q

what gene is involved when have short broad terminal phalanges on first digits

A

HOXD13

Bradydactyly types D and E

39
Q

what gene is involved in achondroplasia

A

EGR3
short stature
midface deformity

40
Q

genes in osteogenesis imperfecta types 1-4

A

COL1A1
COL1A2
bone fragility

41
Q

what type of collagen is affected in osteogenesis imperfecta

A

type I

42
Q

most common disease of growth place

A

achondroplasia

43
Q

sclera is blue small teeth

A

osteogenesis imperfecta

44
Q

small teeth in OI is from what

A

decreased dentin and poor enamel from dec in collagen I

45
Q

what forms of OI are just less Sx than type I

A

3 and 4

46
Q

type II OI prognoisis

A

usually die in utero

so many fractures

47
Q

marble bone disease

A

osteopetrosis

48
Q

what causes OP

A

reduced bone resoprtion and diffuse skeletal sclerosis from impaired formation and function of osteoclasts

49
Q

what component of bone in OP is overproduced

A

mineral

50
Q

appearance of OP on XR

A

chalk appearing, so white

bowing of long bones

51
Q

BM in OP

A

primary spongiosa fills cavity so no room for blood cells

52
Q

what aprt of long bone is most affected in OP

A

metaphysis

53
Q

lab findings in OP

A

anemia, thrombocytopenia, leukopenia (infections)

54
Q

findings in infant with OP

A

CN defects from small foramina
infections from leukopenia
prominant HSM from dec BM

55
Q

mutation in OP

A

CA2 metabolic enzyme

56
Q

dysostoses

A

abrnomalities in single bone or localized group of bones

arise from defects in migration and condensation of mesenchyme

57
Q

dysplasia of bone

A

global disorganization of bone and cartilage

Year2 MSK exam 1 (26 decks)

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