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Flashcards in MSK path IV Deck (57)
1

tumor with schwann cell differentiation

peripheral nerve sheath tumor

2

3 common types of peripheral nerve sheath tumor

schwannoma
neurofibroma
malignant peripheral nerve sheath tumor

3

peripheral nerve sheath tumors are assoc with what familial tumor syndromes

NF I, NF II, schwannomatosis

4

what is a consistent finding in all schwannomas

loss of expresion of NF 2 that produces merlin

5

schwannomas

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

6

Antoni A and Antoni B

A- cellular
B- noncellular
schwannoma

7

Verocary bodies

anuclear rows
schwannoma

8

Sx from schwannomas

usually from compression
tinnitus, hearing loss

9

in cranial vault where are schwannomas

cerebellopontine angle where attach to CN VIII

10

acoustic neuroma

schwannoma in CPA

11

What is a neurofibroma

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

12

cell marker for neurofibroma

CD34+

13

pedunculated nodule with CD34+

supergicial cutaneous NF

14

pedunculated nodules all over with CD34+

NF1- associated

15

large plaquelike elevation of skin CD34+

diffuse NF, assoc NF1

16

plexiform neurofibromas

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

17

guaranteed to see what with NF-1

plexiform neurofibromas

18

what are malignant peripheral nerve sheath tumors though to arise from

malignant transformation of plexiform neurofibromas

19

divergent differentiation

focal areas that have other lines of differentiation
glandular, cartilaginous, osseous, rhabdomyoblastic morphology

20

triton tumor

Malignant PNST with divergent differentiation

21

cafe au lait spots

NF 1

22

NF 2

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

23

composition of bone matrix

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

24

what collagen is in bone

type I

25

what regulates synthesis of boney matrix

hormonal and local mediators

26

what indicates inactive osteoblasts

decrease in cytoplasm

27

what are osteocytes

inactive osteoblasts that become embedded in matrix

28

osteocytes help what

control Ca and phosphate levels in microenvironment and detect mechanical forces "mechanotransduction"

29

specialized mutlinucleated macrophages in bone

osteoclasts

30

how do osteoclasts break down bown

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

how many nuclei are in osteoclasts

3-25

32

osteoclast can be mis identified as what

megakaryocyte

33

zones in active bone growth plate

reserve zone
zone of proliferation
zone of hypertrophy
zone of mineralization
primary spongiosa

34

how much of adult skeleton is replaced anually

10%

35

peak bone mass

after cessation of skeletal growth

36

role of OPG

bind up RANKL so osteoclasts cannot be activated

37

what stimulates production RANKL

M-CSF on osteoblast

38

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

HOXD13
Bradydactyly types D and E

39

what gene is involved in achondroplasia

EGR3
short stature
midface deformity

40

genes in osteogenesis imperfecta types 1-4

COL1A1
COL1A2
bone fragility

41

what type of collagen is affected in osteogenesis imperfecta

type I

42

most common disease of growth place

achondroplasia

43

sclera is blue small teeth

osteogenesis imperfecta

44

small teeth in OI is from what

decreased dentin and poor enamel from dec in collagen I

45

what forms of OI are just less Sx than type I

3 and 4

46

type II OI prognoisis

usually die in utero
so many fractures

47

marble bone disease

osteopetrosis

48

what causes OP

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

49

what component of bone in OP is overproduced

mineral

50

appearance of OP on XR

chalk appearing, so white
bowing of long bones

51

BM in OP

primary spongiosa fills cavity so no room for blood cells

52

what aprt of long bone is most affected in OP

metaphysis

53

lab findings in OP

anemia, thrombocytopenia, leukopenia (infections)

54

findings in infant with OP

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

55

mutation in OP

CA2 metabolic enzyme

56

dysostoses

abrnomalities in single bone or localized group of bones
arise from defects in migration and condensation of mesenchyme

57

dysplasia of bone

global disorganization of bone and cartilage