Dougherty Part 7 Flashcards

1
Q

what is the most common cause of soft tissue tumors

A

unknown

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2
Q

what associations are there with soft tissue tumors

A
radiation
trauma (post mastectomy angiosarcoma)
HHV8
Chemical burns
thermal burns
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3
Q

soft tissue tumors metastasize how and to where

A

blood born

lungs, liver, bone

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4
Q

neurofibromatosis type 1 is associated with what

A

malignant peripheral nerve sheath tumor

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5
Q

gardner syndrome is associated with what

A

fibromatosis (desmoid)

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6
Q

li-froumeni syndrome (mutant p53) is associated with what

A

soft tissue sarcoma and other malignancies

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7
Q

hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu Disease) is associated with what

A

telangiectasias over the skin and mucosal surfaces

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8
Q

what is the translation and fusion protein involved in Ewing Sarcoma and primitive neuroectodermal tumor

A

t(11;22)

EWS-FLI1 fusion

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9
Q

what is the translation and fusion protein involved in liposarcoma-myxoid and round cell type

A

t(12;16)

FUS-DDIT3 fusion

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10
Q

what is the translation and fusion protein involved in synovial sarcoma

A

t(X;18)

SS18-SSX fusion gene

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11
Q

what is the translation and fusion protein involved in rhabdomyosarcoma-alveolar type

A

t(2;13)

PAX3-FOX01 fusion

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12
Q

what is the translation and fusion protein involved in extra skeletal mixed chondrosarcoma

A

t(9;22)

EWS-CHN fusion

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13
Q

what is a spindle cell (appearance)

A

rod-shaped, long axis at least twice short axis

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14
Q

spindle cells are associated with what

A

fibrous, schwann cell, fibrohistiocytic, smooth muscle

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15
Q

small round (blue) cells appear how

A

round, little cytoplasm

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16
Q

epithelioid cells appear how

A

polyhedral

more cytoplasm

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17
Q

epithelioid cells are associated with what

A

epithelioid sarcoma

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18
Q

small round (blue) cells are associated with what

A

rhabdomyosarcoma
PNET
Ewings Sarcoma

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19
Q

matrix producing cells are found where

A

bone cartilage

20
Q

matrix producing cells are associated with what

A

extraskeletal osteosarcoma

21
Q

biphasic cells are what

A

spindle and epithelial cells

22
Q

biphasic cells are associated with what

A

biphasic synovial sarcoma

23
Q

fascicles of spindles are seen in what

A

smooth muscle

24
Q

short fascicles of spindle cells radiating form center (storeroom; pinwheel-like) is seen in what

A

fibrohistiocytic

25
Q

nuclei in columns (palisading) is seen in what

A

schwann cell

26
Q

herringbone pattern is seen in what

A

fibrosarcoma

27
Q

biphasic: spindle and epithelioid cells are seen in what

A

synovial sarcoma

28
Q

what is the most common adult soft tissue tumor

A

lipoma

29
Q

a small 2cm mass on back or arm is most likely what

A

lipoma

30
Q

lipomas are normally painless with what exception

A

angiolipoma

31
Q

what are the most common locations of lipomas

A

subcutaneous trunk and proximal extremities

32
Q

what is the conventional appearance of a lipoma

A

thinly encapsulated yellow tumor

lobules of mature fat

33
Q

what is a lipoma of fibrous areas

A

fibrolipoma

34
Q

what is a lipoma of small vessels

A

angiolipoma

35
Q

what is a lipoma of spindle cell areas

A

spindle cell lipoma

36
Q

what is a lipoma within a muscle

A

intramuscular lipoma

37
Q

liposarcoma is common in what patients

A

adults 40-60

38
Q

where are liposarcomas commonly found

A

deep soft tissues:
retroperitoneum (often very large)
proximal extremities

39
Q

what types of liposarcomas are there

A

well-differentiated (retroperitoneal)
myxoid/round cell (extremities)
pleomorphic-very aggressive course
dedifferentiated

40
Q

what is the most common type of liposarcoma

A

well differentiated

41
Q

what is a lipoblast and when is it seen

A

cytoplasmic vacuoles scallop nucleus

seen in well differentiated liposarcoma

42
Q

what is seen microscopically in a well differentiated liposarcoma

A

atypical spindle cells

lipoblasts

43
Q

well differentiated liposarcomas are due to what

A

MDM2 amplification (inhibits p53)

44
Q

MDM2 amplification is seen in what

A

well differentiated liposarcoma

45
Q

when does one see scalloped nuclei microscopically

A

well differentiated liposarcoma