Dougherty Part 8

Question 1

myxoid/round cell liposarcomas are due to what

Answer 1

t(12;16) FUS-DDIT3 fusion protein

Question 2

where are mixed/round cell liposarcomas found

Answer 2

intramuscular

most often THIGH

Question 3

myxoid liposarcoma have what appearance microscopically

Answer 3

chicken wire pattern of thin capillaries

rare lipoblasts

Question 4

what liposarcoma is very aggressive

Answer 4

pleomorphic liposarcoma

Question 5

what liposarcoma is a sarcoma arising from a well-differnetiated liposarcoma

Answer 5

dedifferentiated liposarcoma

Question 6

what fibrous (myofibroblastic) tumors/proliferations are there (overall categories)

Answer 6

benign reactive proliferations
fibromatoses
fibroma
fibrosarcoma

Question 7

what are the benign reactive fibrous proliferations

Answer 7

nodular fasciitis

myositis ossificans

Question 8

what are the fibromatoses

Answer 8

deep fibrzomatosis (Desmoid)
superficial fibromatosis

Question 9

what is the most common location of nodular fasciitis

Answer 9

volvar forearm

Question 10

nodular fasciitis is a proliferation of what

Answer 10

spindle cell proliferation of fibroblasts

Question 11

what is commonly found on the volvar forearm

Answer 11

nodular faciitis

Question 12

what does nodular fasciitis present as

Answer 12

solitary rapidly growing 2-3cm
may be painful
10% history of trauma

Question 13

nodular fasciitis is found in what tissue

Answer 13

deep dermis, subcutis, or muscle

Question 14

nodular fasciitis is what type of process

Answer 14

reactive process

rarely recurs if excised

Question 15

myositis/panniculitis ossificans is found in what type of patients

Answer 15

athletic adolescents and young adults

50% trauma

Question 16

in what tissues is myositis/panniculitis ossificans found

Answer 16

subcutaneous and muscle

Question 17

how does myositis/panniculitis ossificans present

Answer 17

early painful
circumscribed and firm
3-6cm

Question 18

what is the end result of myositis/panniculitis ossificans

Answer 18

trabecular bone with marrow

Question 19

what are the superficial fibromatoses

Answer 19

palmer fibromatosis
plantar fibromatosis
penile fibromatosis

Question 20

what are the deep-seated fibromatoses (desmoid tumor)

Answer 20

extra-abdominal
abdominal
intra-abdomina

Question 21

nodular or poorly defined fascicles of fibroblasts and abundant collagen are seen in what

Answer 21

superficial fibromatosis

Question 22

dupuytren’s contracture is what

Answer 22

palmar fibromatosis
nodular thickening of palmar facia
puckers skin and digit flexion contraction (mostly 4 and 5)
50% bilateral

Question 23

what is plantar fibromatosis

Answer 23

irregular nodular thickening of plantar fascia

Question 24

peyronie’s disease is what

Answer 24

penile fibromatosis
induration or mass on dorsolateral penis
can cause abnormal curvature of penis
can constrict urethra

Question 25

deep-seated fibromatosis (desmoid tumors) are due to what

Answer 25

mutations in APC or beta-catenin

Question 26

when do deep-seated fibromatosis (desmoid tumors) recur

Answer 26

if not completely excised (need wide margin)

Question 27

when do deep-seated fibromatosis (desmoid tumors) present

Answer 27

teens-30s

Question 28

where are abdominal deep-seated fibromatosis found and in what patients

Answer 28

anterior abdominal wall | women during or after pregnancy or C-section

Question 29

where are intra-abdominal deep-seated fibromatosis found and in what patients

Answer 29

mesentery and pelvic walls | Gardner Syndrome

Question 30

what do deep-seated fibromatosis present as

Answer 30

gray-white poorly demarcated | fibroblastic in fascicles infiltrate tissue

Question 31

spindle cell with areas of herringbone pattern are seen in what

Answer 31

fibrosarcoma

Question 32

where does fibrosarcoma present

Answer 32

most common in deep extremities

Question 33

what presents an infiltrative fish-flesh with hemorrhage and necrosis

Answer 33

fibrosarcoma

Question 34

what is the most common soft tissue sarcoma of childhood and adolescence

Answer 34

rhabdomyosarcoma

Question 35

where does rhabdomyosarcoma typically present

Answer 35

head/neck (nasal cavity, orbit, middle ear)

Question 36

60% of rhabdomyosarcoma are what

Answer 36

embryonal rhabdomyosarcoma | in children under 10

Question 37

sarcoma botryoides is a variant of what

Answer 37

embryonal rhabdomyosarcoma

Question 38

what is seen in sarcoma botryoides variant of embryonal rhabdomyosarcoma

Answer 38

"cluster of grapes" protrude into lumen cambium layer: submucosal hypercellular area best prognosis amongst embryonal rhabdomyosarcoma

Question 39

where are sarcoma botryoides usually found

Answer 39

midline structures: bladder, vagina

Question 40

what is seen in embryonal rhabdomyosarcoma

Answer 40

soft gray infiltrative mass mimic skeletal msucle embryogenesis round and spindle cells

Question 41

what are the myogenic markers seen in rhabdomyoblast

Answer 41

MYOD1 myogenin desmin

Question 42

what is a rhabdomyoblast

Answer 42

eccentric eosinophilic cytoplasm with thick and thin filaments tadpole cells and strap cells EM: sarcomeres

Question 43

what is seen histologically in rhabdomyosarcoma

Answer 43

rhabdomyoblast small round blue cells may see cross striations in some cells