Soft Tissue Flashcards
what is more common in soft tissue cancers, benign or malignant
benign
soft tissue cancers metastasize to what and through what
lungs
liver
bone
-blood
how is bone involved in soft tissue cancers
direct extension
metastasis
what is a classic associated with the etiology of soft tissue cancers
radiation
Translocation and gene problem: Ewing sarcoma and primitive neuroectodermal tumor
t(11:22)
EWS-FLI1 fusion
Translocation and gene problem: liposarcoma-myxoid and found cell type
t(12:16)
FUS-DDIT3 fusion
Translocation and gene problem: Synovial sarcoma
t(X:18)
SS18-SSX fusion gene
Translocation and gene problem: Rhabdomyosarcoma-alveolar type
t(2:13)
PAX3-FOX01 fusion
Translocation and gene problem: extraskeletal myxoid chondrasarcoma
t(9:22)
EWS-CHN fusion
Rhabdomyosarcoma
malignant tumor involving striated muscle tissue
shape of spindle cell
rod-shaped
- long axis at least twich short axis
shape of small round (blue) cells
Round
little cytoplasm
shape of epithelioid cells
polyhedral; more cytoplasm
where are matrix producing cells found
bone and cartilage
biphasic cell is combination of what cell types
spindle and epithelial cells
Spindle cells found in what cancer
fibrous
schwann cell
fibrohistiocytic
smooth muscle
wha is small round ( blue ) cell found in what cancer
rhabdomyosarcoma
PNET/Ewing
what is Epitheloid sarcoma found in what cancer
Epithelioid sarcoma
matrix producing cells found in what cancer
extraskelatal osteosarcoma
Biphasic cell is found in what cancer
biphasic synovial sarcoma
Herringbone pattern is found in what cancer
Fibrosarcoma
Nuclei in columns ( palisading) pattern found in what cancer
Schwann cell
Fasicicles of spindle cells pattern found in
smooth muscles
short fascicles of spindle cells radiating from center ( storiform; pinwheel-like) pattern found in
fibrohistiocytic
what is the most common adult soft tissue tumor
lipoma
Is there pain associated with lipoma? what’s the exception
no
angiolipoma
what is the most common location of lipoma
subcutaneous trunk and proximal extremeties
location of fibrolipoma
fibrous area
location of angiolipoma
small vessels
where are liposarcoma’s usually located
deep soft tissue
- retroperitoneum
- promixal extremities
what is the most common type of liposarcoma
well- differentiated
well- differentiated liposarcoma has a similar gross appearance as what
benign lipoma
what is histo characteristic of well- differentiated liposarcoma
aytpical spindle cells
-cytoplasmic vacuoles scallop nucleus
what gene does well- differentiated liposarcoma amplify? what does this cause
MDM2
-inhibits p53
myxoid/round cell Liposarcoma is what translocation and gene
t(12;16)
FUS-DDIT3 fusion protein
where do myxoid/round cell liposarcoma usually occur on the body
intramuscular; thigh
what is the histo characterstic of myxoid/round cell liposarcoma
chicken wire pattern of thin capillaries
- rare lipoblast
how aggressive is pleomorphic liposarcoma
very
dedifferentiated liposarcoma arises from what
well-differentiated liposarcoma
what is the most common volvar forearm
Nodular Fasiitis
what layers of the body does Nodular Fasciitis use
Deep dermis, subcutis or muscle
what type of cells proliferate in nodular fasciits
spindle cell proliferation of fibroblasts
Does nodular Fasciitis reaccur
no if excised
how big and fast can nodular fasciitis get
rapid growing up to 2-3cm
who usually gets Myositis/Panniculitis Ossificans
athletic adolescents and young adults
what layers of skin are involved for Myositis/Panniculitis Ossificans
subcutaneous and muscle
Fibromatoses is subdivided into what
superficial fibromatosis
deep-seated fibromatosis ( desmoid tumor)
what are superficial fibromatosis made of
nodular or poorly defined fasciciles of fibroblasts and abundant collagen
after excision can superficial fibromatosis come back
may recur
superficial fibromatosis: Palmer Fibromatosis ( Dupuytren’s contracture) what is it
nodular thickening of palmer fascia
- puckers skin and digit flexion contraction
- bilateral
- 4th and 5th digit
superficial fibromatosis: plantar fibromatosis what is it
irregular of nodular thickening of plantar fascia
-bilateral infrequent
superficial fibromatosis: penile Fibromatosis another name
Peyronie’s disease
superficial fibromatosis: penile Fibromatosis what is it
induration or mass on dorsolateral penis
-abnormal curvature of penis
constricts urethra
superficial fibromatosis: Palmer Fibromatosis other name
Dupuytren’s contracture
another name for deep-seated Fibromatosis
Desmoid tumors
Desmoid tumors have mutations in what
APC or Beta-Catenin
how large, location and metastasize ability of Deep-seated fibromatosis
large
locally aggressive
do not metastasize
can desmoid tumors recur
not if excised, need big margin
what are the 3 types of deep-seated fibromatosis
abdominal
intra-abdominal
extra-abdominal
where does abdominal deep-seated fibromatosis occur? who usually gets them
anterior abdominal wall
women during or after pregnancy or C-section
Where does Intra-abdominal desmoid occur and in what syndrome
Mesentry and pelvic walls
Gardner Syndrome
what color are deep-seated fibromatosis
gray-white poorly demarcated
what do fibroblasts is fascicles infiltrate in Deep-seated fibromatosis
infilterate tissue
how often doe Fibrosarcoma occur
rare
where does fibrosarcoma occur
deep extremities
what type of cells are found in Fibrosarcoma? what pattern do they form
spindle cells with Herringbone pattern
name 3 types of skeletal muscle tumors
Rhabdomyoma
Cardiac Rhabdomyoma
Rhabdomyosarcoma
what is the most common soft tissue sarcoma of childhood and adolescence
Rhabdomyosarcoma
where does Rhabdomyosarcoma foundon the body
head/neck
GI tract
what is a subtype of Embryonal Rhabdomysosarcoma
Sarcoma Botryoides: in walls of hollow viscera and mucusal lined structures
Embryonal Rhabdomyosarcoma has what colored mass
soft gray infiltrative
Embryonal Rhabdomysarcoma mimics what
skeletal muscle embryogenesis: round and spindle cells
Sarcoma Botryoides describe it
cluster of grapes
cambium layer: submuscoasl hyercellular layer
Characterize the Rhabdomyosarcoma cells
Eccentric eosinophilic cytoplasm with thick and thin filaments
- tadpole cells and strap cells
- MYOD1, myogen, desmin
Small round blue cells
who usually gets alveolar rhabdomyosarcoma
early to mid-adolescence
where does alveolar rhabdomyosarcoma occur
deep muscles of extremities
why is it called alveolar Rhabdomyosarcoma
fibrous setpa divide tumor into alveolar like spaces
translocation for Alveolar Rhabdomyosarcoma
t(2;13) and t (1;13)
PAX3 or PAX7 to FOXO1
pleomorphic Rhabdomyosarcoma occurs in who and where
adults deep soft tissue
what are two diagnostic features of pleomorphic Rhabdomyosarcoma
desmin positive
MyoD1 or myogenin positive
rate the Rhabdomyosarcoma prognosis
Sarcoma: best
Embryonal: second best
Alveolar/pleomorhic: poor
who has better prognosis with Rhabdomyosarcoma
children better than adults
what is leiomyoma
benign smooth muscle tumor
where does Leiomyoma usually occur
uterus
where does Leiomyoma occur on the skin
erector pili
nipples
scrotum
labia
what does Leiomyoma do to smooth muscles
bland smooth muscle cells in fascicles
what gender gets leiomyosarcoma
females
what does Leiomyosarcoma look like
white mases
what cells are ound in leiomyosarcoma
malignant spindle cells
where does Leiomyosarcoma impact
skin
deep extrememites
retroperitoneum
what is Leiomyosarcoma negative for
MyoD1 and myogenin
where does synovial sarcoma occur
deep and around large joints
translocation for synovial sarcoma
t(X;18) SS18-SSX1 or SS18-SSX2
what are two types of synovial sarcoma
biphasic dual differentiation
monophasic ( spindle cell) variant
biphasic occurs where and has what type of cell
epithelial cells as glands, cords, or nests
spindle cells: cellular in fascicles
monophasic spindle cell variant is positive for what
cytokertins
epithelial membrane antigen
another name for undifferentiated pleomorphic sarcoma
malignant fibrous histiocytoma
what is malignant fibrous histiocytoma
group of aggressive neoplasms
where does malignant fibrous histiocytoma occur
proximal extremeites and retroperitoneum
what does malignant fibrous histiocytoma look like
large gray, hemorrhagic and necrotic
Epithelial membrane antigen and cytokeratin
general epithelial marker
MyoD1 and Myogenin
skeletal muslce
actin and desmin
skeletal or smooth muscle
vemintin
nonepithelial tumors
smooth muscle actin
smooth muscle
Epithelium
cytokeratin and EMA
skeletal muscle
MyoD1
myogenin
desmin
actin
smooth muscle
desmin
actin
nonepithelial tumors
vimentin
spindle cell benign
nodular fasciitis fibrous histiocytoma fibromatosis myositis ossificans leiomyoma
spindle cell malignant
fibrosarcoma
leiomyosarcoma
undifferentiated pleomorphic sarcoma
small round blue cells benign
lymphoid hyperplasia
small round blue cells malignant
rhabdomyosarcoma
round cell liposarcoma
lymphoma
biphasic
synovial sarcoma
