MSK path IV

Question 1

tumor with schwann cell differentiation

Answer 1

peripheral nerve sheath tumor

Question 2

3 common types of peripheral nerve sheath tumor

Answer 2

schwannoma
neurofibroma
malignant peripheral nerve sheath tumor

Question 3

peripheral nerve sheath tumors are assoc with what familial tumor syndromes

Answer 3

NF I, NF II, schwannomatosis

Question 4

what is a consistent finding in all schwannomas

Answer 4

loss of expresion of NF 2 that produces merlin

Question 5

schwannomas

Answer 5

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

Question 6

Antoni A and Antoni B

Answer 6

A- cellular
B- noncellular
schwannoma

Question 7

Verocary bodies

Answer 7

anuclear rows

schwannoma

Question 8

Sx from schwannomas

Answer 8

usually from compression

tinnitus, hearing loss

Question 9

in cranial vault where are schwannomas

Answer 9

cerebellopontine angle where attach to CN VIII

Question 10

acoustic neuroma

Answer 10

schwannoma in CPA

Question 11

What is a neurofibroma

Answer 11

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

Question 12

cell marker for neurofibroma

Answer 12

CD34+

Question 13

pedunculated nodule with CD34+

Answer 13

supergicial cutaneous NF

Question 14

pedunculated nodules all over with CD34+

Answer 14

NF1- associated

Question 15

large plaquelike elevation of skin CD34+

Answer 15

diffuse NF, assoc NF1

Question 16

plexiform neurofibromas

Answer 16

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

Question 17

guaranteed to see what with NF-1

Answer 17

plexiform neurofibromas

Question 18

what are malignant peripheral nerve sheath tumors though to arise from

Answer 18

malignant transformation of plexiform neurofibromas

Question 19

divergent differentiation

Answer 19

focal areas that have other lines of differentiation

glandular, cartilaginous, osseous, rhabdomyoblastic morphology

Question 20

triton tumor

Answer 20

Malignant PNST with divergent differentiation

Question 21

cafe au lait spots

Answer 21

NF 1

Question 22

NF 2

Answer 22

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

Question 23

composition of bone matrix

Answer 23

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

Question 24

what collagen is in bone

Answer 24

type I

Question 25

what regulates synthesis of boney matrix

Answer 25

hormonal and local mediators

Question 26

what indicates inactive osteoblasts

Answer 26

decrease in cytoplasm

Question 27

what are osteocytes

Answer 27

inactive osteoblasts that become embedded in matrix

Question 28

osteocytes help what

Answer 28

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

Question 29

specialized mutlinucleated macrophages in bone

Answer 29

osteoclasts

Question 30

how do osteoclasts break down bown

Answer 30

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

Question 31

how many nuclei are in osteoclasts

Answer 31

3-25

Question 32

osteoclast can be mis identified as what

Answer 32

megakaryocyte

Question 33

zones in active bone growth plate

Answer 33

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

Question 34

how much of adult skeleton is replaced anually

Answer 34

10%

Question 35

peak bone mass

Answer 35

after cessation of skeletal growth

Question 36

role of OPG

Answer 36

bind up RANKL so osteoclasts cannot be activated

Question 37

what stimulates production RANKL

Answer 37

M-CSF on osteoblast

Question 38

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

Answer 38

HOXD13 | Bradydactyly types D and E

Question 39

what gene is involved in achondroplasia

Answer 39

EGR3 short stature midface deformity

Question 40

genes in osteogenesis imperfecta types 1-4

Answer 40

COL1A1 COL1A2 bone fragility

Question 41

what type of collagen is affected in osteogenesis imperfecta

Answer 41

type I

Question 42

most common disease of growth place

Answer 42

achondroplasia

Question 43

sclera is blue small teeth

Answer 43

osteogenesis imperfecta

Question 44

small teeth in OI is from what

Answer 44

decreased dentin and poor enamel from dec in collagen I

Question 45

what forms of OI are just less Sx than type I

Answer 45

3 and 4

Question 46

type II OI prognoisis

Answer 46

usually die in utero | so many fractures

Question 47

marble bone disease

Answer 47

osteopetrosis

Question 48

what causes OP

Answer 48

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

Question 49

what component of bone in OP is overproduced

Answer 49

mineral

Question 50

appearance of OP on XR

Answer 50

chalk appearing, so white | bowing of long bones

Question 51

BM in OP

Answer 51

primary spongiosa fills cavity so no room for blood cells

Question 52

what aprt of long bone is most affected in OP

Answer 52

metaphysis

Question 53

lab findings in OP

Answer 53

anemia, thrombocytopenia, leukopenia (infections)

Question 54

findings in infant with OP

Answer 54

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

Question 55

mutation in OP

Answer 55

CA2 metabolic enzyme

Question 56

dysostoses

Answer 56

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

Question 57

dysplasia of bone

Answer 57

global disorganization of bone and cartilage