Dougherty Part 7 Flashcards
what is the most common cause of soft tissue tumors
unknown
what associations are there with soft tissue tumors
radiation trauma (post mastectomy angiosarcoma) HHV8 Chemical burns thermal burns
soft tissue tumors metastasize how and to where
blood born
lungs, liver, bone
neurofibromatosis type 1 is associated with what
malignant peripheral nerve sheath tumor
gardner syndrome is associated with what
fibromatosis (desmoid)
li-froumeni syndrome (mutant p53) is associated with what
soft tissue sarcoma and other malignancies
hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu Disease) is associated with what
telangiectasias over the skin and mucosal surfaces
what is the translation and fusion protein involved in Ewing Sarcoma and primitive neuroectodermal tumor
t(11;22)
EWS-FLI1 fusion
what is the translation and fusion protein involved in liposarcoma-myxoid and round cell type
t(12;16)
FUS-DDIT3 fusion
what is the translation and fusion protein involved in synovial sarcoma
t(X;18)
SS18-SSX fusion gene
what is the translation and fusion protein involved in rhabdomyosarcoma-alveolar type
t(2;13)
PAX3-FOX01 fusion
what is the translation and fusion protein involved in extra skeletal mixed chondrosarcoma
t(9;22)
EWS-CHN fusion
what is a spindle cell (appearance)
rod-shaped, long axis at least twice short axis
spindle cells are associated with what
fibrous, schwann cell, fibrohistiocytic, smooth muscle
small round (blue) cells appear how
round, little cytoplasm
epithelioid cells appear how
polyhedral
more cytoplasm
epithelioid cells are associated with what
epithelioid sarcoma
small round (blue) cells are associated with what
rhabdomyosarcoma
PNET
Ewings Sarcoma
matrix producing cells are found where
bone cartilage
matrix producing cells are associated with what
extraskeletal osteosarcoma
biphasic cells are what
spindle and epithelial cells
biphasic cells are associated with what
biphasic synovial sarcoma
fascicles of spindles are seen in what
smooth muscle
short fascicles of spindle cells radiating form center (storeroom; pinwheel-like) is seen in what
fibrohistiocytic
nuclei in columns (palisading) is seen in what
schwann cell
herringbone pattern is seen in what
fibrosarcoma
biphasic: spindle and epithelioid cells are seen in what
synovial sarcoma
what is the most common adult soft tissue tumor
lipoma
a small 2cm mass on back or arm is most likely what
lipoma
lipomas are normally painless with what exception
angiolipoma
what are the most common locations of lipomas
subcutaneous trunk and proximal extremities
what is the conventional appearance of a lipoma
thinly encapsulated yellow tumor
lobules of mature fat
what is a lipoma of fibrous areas
fibrolipoma
what is a lipoma of small vessels
angiolipoma
what is a lipoma of spindle cell areas
spindle cell lipoma
what is a lipoma within a muscle
intramuscular lipoma
liposarcoma is common in what patients
adults 40-60
where are liposarcomas commonly found
deep soft tissues:
retroperitoneum (often very large)
proximal extremities
what types of liposarcomas are there
well-differentiated (retroperitoneal)
myxoid/round cell (extremities)
pleomorphic-very aggressive course
dedifferentiated
what is the most common type of liposarcoma
well differentiated
what is a lipoblast and when is it seen
cytoplasmic vacuoles scallop nucleus
seen in well differentiated liposarcoma
what is seen microscopically in a well differentiated liposarcoma
atypical spindle cells
lipoblasts
well differentiated liposarcomas are due to what
MDM2 amplification (inhibits p53)
MDM2 amplification is seen in what
well differentiated liposarcoma
when does one see scalloped nuclei microscopically
well differentiated liposarcoma
