Dougherty Part 8 Flashcards
myxoid/round cell liposarcomas are due to what
t(12;16) FUS-DDIT3 fusion protein
where are mixed/round cell liposarcomas found
intramuscular
most often THIGH
myxoid liposarcoma have what appearance microscopically
chicken wire pattern of thin capillaries
rare lipoblasts
what liposarcoma is very aggressive
pleomorphic liposarcoma
what liposarcoma is a sarcoma arising from a well-differnetiated liposarcoma
dedifferentiated liposarcoma
what fibrous (myofibroblastic) tumors/proliferations are there (overall categories)
benign reactive proliferations
fibromatoses
fibroma
fibrosarcoma
what are the benign reactive fibrous proliferations
nodular fasciitis
myositis ossificans
what are the fibromatoses
deep fibrzomatosis (Desmoid) superficial fibromatosis
what is the most common location of nodular fasciitis
volvar forearm
nodular fasciitis is a proliferation of what
spindle cell proliferation of fibroblasts
what is commonly found on the volvar forearm
nodular faciitis
what does nodular fasciitis present as
solitary rapidly growing 2-3cm
may be painful
10% history of trauma
nodular fasciitis is found in what tissue
deep dermis, subcutis, or muscle
nodular fasciitis is what type of process
reactive process
rarely recurs if excised
myositis/panniculitis ossificans is found in what type of patients
athletic adolescents and young adults
50% trauma
in what tissues is myositis/panniculitis ossificans found
subcutaneous and muscle
how does myositis/panniculitis ossificans present
early painful
circumscribed and firm
3-6cm
what is the end result of myositis/panniculitis ossificans
trabecular bone with marrow
what are the superficial fibromatoses
palmer fibromatosis
plantar fibromatosis
penile fibromatosis
what are the deep-seated fibromatoses (desmoid tumor)
extra-abdominal
abdominal
intra-abdomina
nodular or poorly defined fascicles of fibroblasts and abundant collagen are seen in what
superficial fibromatosis
dupuytren’s contracture is what
palmar fibromatosis
nodular thickening of palmar facia
puckers skin and digit flexion contraction (mostly 4 and 5)
50% bilateral
what is plantar fibromatosis
irregular nodular thickening of plantar fascia
peyronie’s disease is what
penile fibromatosis
induration or mass on dorsolateral penis
can cause abnormal curvature of penis
can constrict urethra
deep-seated fibromatosis (desmoid tumors) are due to what
mutations in APC or beta-catenin
when do deep-seated fibromatosis (desmoid tumors) recur
if not completely excised (need wide margin)
when do deep-seated fibromatosis (desmoid tumors) present
teens-30s
where are abdominal deep-seated fibromatosis found and in what patients
anterior abdominal wall
women during or after pregnancy or C-section
where are intra-abdominal deep-seated fibromatosis found and in what patients
mesentery and pelvic walls
Gardner Syndrome
what do deep-seated fibromatosis present as
gray-white poorly demarcated
fibroblastic in fascicles infiltrate tissue
spindle cell with areas of herringbone pattern are seen in what
fibrosarcoma
where does fibrosarcoma present
most common in deep extremities
what presents an infiltrative fish-flesh with hemorrhage and necrosis
fibrosarcoma
what is the most common soft tissue sarcoma of childhood and adolescence
rhabdomyosarcoma
where does rhabdomyosarcoma typically present
head/neck (nasal cavity, orbit, middle ear)
60% of rhabdomyosarcoma are what
embryonal rhabdomyosarcoma
in children under 10
sarcoma botryoides is a variant of what
embryonal rhabdomyosarcoma
what is seen in sarcoma botryoides variant of embryonal rhabdomyosarcoma
“cluster of grapes” protrude into lumen
cambium layer: submucosal hypercellular area
best prognosis amongst embryonal rhabdomyosarcoma
where are sarcoma botryoides usually found
midline structures: bladder, vagina
what is seen in embryonal rhabdomyosarcoma
soft gray infiltrative mass
mimic skeletal msucle embryogenesis
round and spindle cells
what are the myogenic markers seen in rhabdomyoblast
MYOD1
myogenin
desmin
what is a rhabdomyoblast
eccentric eosinophilic cytoplasm with thick and thin filaments
tadpole cells and strap cells
EM: sarcomeres
what is seen histologically in rhabdomyosarcoma
rhabdomyoblast
small round blue cells
may see cross striations in some cells
