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Bone > Dougherty Part 8 > Flashcards

Dougherty Part 8 Flashcards

1
Q

myxoid/round cell liposarcomas are due to what

A

t(12;16) FUS-DDIT3 fusion protein

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2
Q

where are mixed/round cell liposarcomas found

A

intramuscular

most often THIGH

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3
Q

myxoid liposarcoma have what appearance microscopically

A

chicken wire pattern of thin capillaries

rare lipoblasts

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4
Q

what liposarcoma is very aggressive

A

pleomorphic liposarcoma

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5
Q

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

A

dedifferentiated liposarcoma

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6
Q

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

A

benign reactive proliferations
fibromatoses
fibroma
fibrosarcoma

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7
Q

what are the benign reactive fibrous proliferations

A

nodular fasciitis

myositis ossificans

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8
Q

what are the fibromatoses

A 
deep fibrzomatosis (Desmoid)
superficial fibromatosis
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9
Q

what is the most common location of nodular fasciitis

A

volvar forearm

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10
Q

nodular fasciitis is a proliferation of what

A

spindle cell proliferation of fibroblasts

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11
Q

what is commonly found on the volvar forearm

A

nodular faciitis

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12
Q

what does nodular fasciitis present as

A

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

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13
Q

nodular fasciitis is found in what tissue

A

deep dermis, subcutis, or muscle

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14
Q

nodular fasciitis is what type of process

A

reactive process

rarely recurs if excised

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15
Q

myositis/panniculitis ossificans is found in what type of patients

A

athletic adolescents and young adults

50% trauma

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16
Q

in what tissues is myositis/panniculitis ossificans found

A

subcutaneous and muscle

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17
Q

how does myositis/panniculitis ossificans present

A

early painful
circumscribed and firm
3-6cm

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18
Q

what is the end result of myositis/panniculitis ossificans

A

trabecular bone with marrow

19
Q

what are the superficial fibromatoses

A

palmer fibromatosis
plantar fibromatosis
penile fibromatosis

20
Q

what are the deep-seated fibromatoses (desmoid tumor)

A

extra-abdominal
abdominal
intra-abdomina

21
Q

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

A

superficial fibromatosis

22
Q

dupuytren’s contracture is what

A

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

23
Q

what is plantar fibromatosis

A

irregular nodular thickening of plantar fascia

24
Q

peyronie’s disease is what

A

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

25
Q

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

A

mutations in APC or beta-catenin

26
Q

when do deep-seated fibromatosis (desmoid tumors) recur

A

if not completely excised (need wide margin)

27
Q

when do deep-seated fibromatosis (desmoid tumors) present

A

teens-30s

28
Q

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

A

anterior abdominal wall

women during or after pregnancy or C-section

29
Q

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

A

mesentery and pelvic walls

Gardner Syndrome

30
Q

what do deep-seated fibromatosis present as

A

gray-white poorly demarcated

fibroblastic in fascicles infiltrate tissue

31
Q

spindle cell with areas of herringbone pattern are seen in what

A

fibrosarcoma

32
Q

where does fibrosarcoma present

A

most common in deep extremities

33
Q

what presents an infiltrative fish-flesh with hemorrhage and necrosis

A

fibrosarcoma

34
Q

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

A

rhabdomyosarcoma

35
Q

where does rhabdomyosarcoma typically present

A

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

36
Q

60% of rhabdomyosarcoma are what

A

embryonal rhabdomyosarcoma

in children under 10

37
Q

sarcoma botryoides is a variant of what

A

embryonal rhabdomyosarcoma

38
Q

what is seen in sarcoma botryoides variant of embryonal rhabdomyosarcoma

A

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

39
Q

where are sarcoma botryoides usually found

A

midline structures: bladder, vagina

40
Q

what is seen in embryonal rhabdomyosarcoma

A

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

41
Q

what are the myogenic markers seen in rhabdomyoblast

A

MYOD1
myogenin
desmin

42
Q

what is a rhabdomyoblast

A

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

43
Q

what is seen histologically in rhabdomyosarcoma

A

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

Bone (19 decks)

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