Dougherty Part 7

Question 1

what is the most common cause of soft tissue tumors

Answer 1

unknown

Question 2

what associations are there with soft tissue tumors

Answer 2

radiation
trauma (post mastectomy angiosarcoma)
HHV8
Chemical burns
thermal burns

Question 3

soft tissue tumors metastasize how and to where

Answer 3

blood born

lungs, liver, bone

Question 4

neurofibromatosis type 1 is associated with what

Answer 4

malignant peripheral nerve sheath tumor

Question 5

gardner syndrome is associated with what

Answer 5

fibromatosis (desmoid)

Question 6

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

Answer 6

soft tissue sarcoma and other malignancies

Question 7

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

Answer 7

telangiectasias over the skin and mucosal surfaces

Question 8

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

Answer 8

t(11;22)

EWS-FLI1 fusion

Question 9

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

Answer 9

t(12;16)

FUS-DDIT3 fusion

Question 10

what is the translation and fusion protein involved in synovial sarcoma

Answer 10

t(X;18)

SS18-SSX fusion gene

Question 11

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

Answer 11

t(2;13)

PAX3-FOX01 fusion

Question 12

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

Answer 12

t(9;22)

EWS-CHN fusion

Question 13

what is a spindle cell (appearance)

Answer 13

rod-shaped, long axis at least twice short axis

Question 14

spindle cells are associated with what

Answer 14

fibrous, schwann cell, fibrohistiocytic, smooth muscle

Question 15

small round (blue) cells appear how

Answer 15

round, little cytoplasm

Question 16

epithelioid cells appear how

Answer 16

polyhedral

more cytoplasm

Question 17

epithelioid cells are associated with what

Answer 17

epithelioid sarcoma

Question 18

small round (blue) cells are associated with what

Answer 18

rhabdomyosarcoma
PNET
Ewings Sarcoma

Question 19

matrix producing cells are found where

Answer 19

bone cartilage

Question 20

matrix producing cells are associated with what

Answer 20

extraskeletal osteosarcoma

Question 21

biphasic cells are what

Answer 21

spindle and epithelial cells

Question 22

biphasic cells are associated with what

Answer 22

biphasic synovial sarcoma

Question 23

fascicles of spindles are seen in what

Answer 23

smooth muscle

Question 24

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

Answer 24

fibrohistiocytic

Question 25

nuclei in columns (palisading) is seen in what

Answer 25

schwann cell

Question 26

herringbone pattern is seen in what

Answer 26

fibrosarcoma

Question 27

biphasic: spindle and epithelioid cells are seen in what

Answer 27

synovial sarcoma

Question 28

what is the most common adult soft tissue tumor

Answer 28

lipoma

Question 29

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

Answer 29

lipoma

Question 30

lipomas are normally painless with what exception

Answer 30

angiolipoma

Question 31

what are the most common locations of lipomas

Answer 31

subcutaneous trunk and proximal extremities

Question 32

what is the conventional appearance of a lipoma

Answer 32

thinly encapsulated yellow tumor | lobules of mature fat

Question 33

what is a lipoma of fibrous areas

Answer 33

fibrolipoma

Question 34

what is a lipoma of small vessels

Answer 34

angiolipoma

Question 35

what is a lipoma of spindle cell areas

Answer 35

spindle cell lipoma

Question 36

what is a lipoma within a muscle

Answer 36

intramuscular lipoma

Question 37

liposarcoma is common in what patients

Answer 37

adults 40-60

Question 38

where are liposarcomas commonly found

Answer 38

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

Question 39

what types of liposarcomas are there

Answer 39

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

Question 40

what is the most common type of liposarcoma

Answer 40

well differentiated

Question 41

what is a lipoblast and when is it seen

Answer 41

cytoplasmic vacuoles scallop nucleus | seen in well differentiated liposarcoma

Question 42

what is seen microscopically in a well differentiated liposarcoma

Answer 42

atypical spindle cells | lipoblasts

Question 43

well differentiated liposarcomas are due to what

Answer 43

MDM2 amplification (inhibits p53)

Question 44

MDM2 amplification is seen in what

Answer 44

well differentiated liposarcoma

Question 45

when does one see scalloped nuclei microscopically

Answer 45

well differentiated liposarcoma