B6-016 Soft Tissue Sarcoma Flashcards
what age group does rhabdomyosarcoma typically present in?
children
what age group does synovial sarcoma typically present in?
young adults
what age group does liposarcoma typically present in?
mid to late adult life
most common soft tissue tumors in adults
lipoma
encapsulated mass of mature fat cells
lipoma
one of the most common sarcomas in adults
liposarcoma
caused by amplification of the MDM2 gene
well differentiated/defifferentiated liposarcoma
chromosomal translocation t(12, 16)(q13;11)
fusion of DDIT3(CHOP) gene with FUS gene
mixoid/round cell liposarcoma
cytoplasmic vacuoles of lipid that scallop the nucleus
liposarcoma
lipoblasts and MDM2 amplification
well differentiated liposarcoma
t(12;16)(q13;11) tranlocation
DDIT3-FUS fusion
myxoid liposarcoma
translocation associated with myxoid/round cell liposarcoma
t(12;16)(q13;11)
DDIT3-FUS fusion
reactive pseudosarcomatous proliferation
nodular fasciitis
acute history of solitary rapidly growing mass often on volar aspect of forearm, chest, or back
nodular fasciitis
plump randomly arranged spindle cells in a myxoid background containing lymphocytes and extravasated RBCs
nodular fasciitis
fibroblasts
myxoid background
extravasated RBCs
nodular fasciitis
superficial fibromatosis occuring in the palm
dupuytren’s
superficial fibromatosis occuring in the plantar foot
ledderhosens
superficial fibromatosis occuring in the penis
peronie’s
nodular or poorly defined bundles of mature fibroblasts surrounded by dense collagen
superficial fibromatosis
collagen
fibroblasts
superficial fibromatosis
caused by mutation in the beta-catenin genes
deep seated fibromatosis
most frequently occur in females teens-30s
deep seated fibromatosis
often occur in patients with Gardner syndrome (variant of familial adenomatous polyposis)
intra-abdominal deep seated fibromatosis
grey white, firm infiltrative mass
deep seated fibromatosis
long fasicles of elongated fibroblasts and collagen fibers
IHC + for beta-catenin
deep seated fibromatosis
fibroblasts
trapped skeletal muscle
deep seated fibromatosis
rare sarcoma occuring mostly in the retroperitoneum, thigh, knee, or distal extremities
fibrosarcoma
malignant spindle cells arranged in herringbone pattern with frequent mitoses and areas of necrosis
fibrosarcoma
very aggressive fibrous tumor with high recurrence and metastasis rate
fibrosarcoma
herring bone pattern
fibrosarcoma
benign tumor of smooth muscle common in the uterus
leiomyoma
intersecting bundles of spindle cells with blunt-ended cigar shaped nuclei
leiomyoma
blunt ended cigar shaped nuclei, no atypia, no mitosis
leiomyoma
associated with smooth muscle actin (SMA), H-Caldesmon
leiomyosarcoma
malignant spindle cells with cigar shaped nuclei, frequent mitoses and necrosis
leiomyosarcoma
smooth muscle tumor with necrosis and hemorrhage
leiomyosarcoma
cigar shaped atypical nuclei
leiomyosarcoma
most common soft tissue sarcoma of children and adolescents
rhabdomyosarcoma
“strap cells” with eccentric nucleus with abundant granular eosinophilic cytoplasm with cross striations
rhabdomyosarcoma
desmin, myoglobin, MyoD1
rhabdomyosarcoma
strap cells
rhabdomyosarcoma
[…] rhabdomyosarcoma
site: head, neck, GU
epidemiology: children
clinical: rapidly growing mass
embryonal rhabdomyosarcoma
[…] rhabdomyosarcoma
site: head, neck, extremities
epidemiology: adolescents, all ages
clinical: rapidly growing mass
alveolar rhabdomyosarcoma
[…] rhabdomyosarcoma
site: extremities
epidemiology: older adults
clinical: rapidly growing mass
pleomorphic rhabdomyosarcoma
sarcoma botryoides “bunch of grapes”
embryonal rhabdomyosarcoma
most frequently occurs in children under 5 yo
develops in mucosal linings of nasopharynx, bile duct, vagina, bladder
embryonal rhabdomyosarcoma
characteristic t(2;13)(q35;14) translocation
PAX-OXO1 translocation
alveolar rhabdomyosarcoma
FISH detects FOXO1 breakpoint
alveolar rhabdomyosarcoma
PCR detects FOXO1-PAX fusion
alveolar rhabdomyosarcoma
in alveolar rhabdomyosarcoma, the chimeric PAX3-FOXO1 protein leads to dysregulation of
skeletal muscle differentiation
high grade small round cell tumor
solid growth pattern with central loss of cohesion
alveolar rhabdomyosarcoma
solid growth pattern
small round cells
loss of cohesion
alveolar rhabdomyosarcoma
list the prognosis of best to worst of the rhabdomyosarcomas
botryoid > embryonal > pleomorphic > alveolar
juvenille […] of the skin of newborns is extremely common
capillary hemangiomas
closely packed aggregates of thin walled capillaries
capillary hemangioma
large cavernous vascular spaces filled with blood
often in liver or other deep structures
cavernous hemangiomas
associated with HHV8
kaposi sarcoma
most aggressive form of kaposi sarcoma
AIDS-associated
most common AIDS associated tumor in US
kaposi sarcoma
3 stages of kaposi sarcoma
patches
plaques
nodules
proliferation of slit-like vascular channels lined by plump spindle cells with extravasated RBCs
kaposi sarcoma