B6-016 Soft Tissue Sarcoma Flashcards
(157 cards)
1
Q
what age group does rhabdomyosarcoma typically present in?
A
children
2
Q
what age group does synovial sarcoma typically present in?
A
young adults
3
Q
what age group does liposarcoma typically present in?
A
mid to late adult life
4
Q
most common soft tissue tumors in adults
A
lipoma
5
Q
encapsulated mass of mature fat cells
A
lipoma
6
Q
one of the most common sarcomas in adults
A
liposarcoma
7
Q
caused by amplification of the MDM2 gene
A
well differentiated/defifferentiated liposarcoma
8
Q
chromosomal translocation t(12, 16)(q13;11)
fusion of DDIT3(CHOP) gene with FUS gene
A
mixoid/round cell liposarcoma
9
Q
cytoplasmic vacuoles of lipid that scallop the nucleus
A
liposarcoma
10
Q
lipoblasts and MDM2 amplification
A
well differentiated liposarcoma
11
Q
t(12;16)(q13;11) tranlocation
DDIT3-FUS fusion
A
myxoid liposarcoma
12
Q
translocation associated with myxoid/round cell liposarcoma
A
t(12;16)(q13;11)
DDIT3-FUS fusion
13
Q
reactive pseudosarcomatous proliferation
A
nodular fasciitis
14
Q
acute history of solitary rapidly growing mass often on volar aspect of forearm, chest, or back
A
nodular fasciitis
15
Q
plump randomly arranged spindle cells in a myxoid background containing lymphocytes and extravasated RBCs
A
nodular fasciitis
16
Q
fibroblasts
myxoid background
extravasated RBCs
A
nodular fasciitis
17
Q
superficial fibromatosis occuring in the palm
A
dupuytren’s
18
Q
superficial fibromatosis occuring in the plantar foot
A
ledderhosens
19
Q
superficial fibromatosis occuring in the penis
A
peronie’s
20
Q
nodular or poorly defined bundles of mature fibroblasts surrounded by dense collagen
A
superficial fibromatosis
21
Q
collagen
fibroblasts
A
superficial fibromatosis
22
Q
caused by mutation in the beta-catenin genes
A
deep seated fibromatosis
23
Q
most frequently occur in females teens-30s
A
deep seated fibromatosis
24
Q
often occur in patients with Gardner syndrome (variant of familial adenomatous polyposis)
A
intra-abdominal deep seated fibromatosis
25
grey white, firm infiltrative mass
deep seated fibromatosis
26
long fasicles of elongated fibroblasts and collagen fibers
IHC + for beta-catenin
deep seated fibromatosis
27
fibroblasts
trapped skeletal muscle
deep seated fibromatosis
28
rare sarcoma occuring mostly in the retroperitoneum, thigh, knee, or distal extremities
fibrosarcoma
29
malignant spindle cells arranged in herringbone pattern with frequent mitoses and areas of necrosis
fibrosarcoma
30
very aggressive fibrous tumor with high recurrence and metastasis rate
fibrosarcoma
31
herring bone pattern
fibrosarcoma
32
benign tumor of smooth muscle common in the uterus
leiomyoma
33
intersecting bundles of spindle cells with blunt-ended cigar shaped nuclei
leiomyoma
34
blunt ended cigar shaped nuclei, no atypia, no mitosis
leiomyoma
35
associated with smooth muscle actin (SMA), H-Caldesmon
leiomyosarcoma
36
malignant spindle cells with cigar shaped nuclei, frequent mitoses and necrosis
leiomyosarcoma
37
smooth muscle tumor with necrosis and hemorrhage
leiomyosarcoma
38
cigar shaped atypical nuclei
leiomyosarcoma
39
most common soft tissue sarcoma of children and adolescents
rhabdomyosarcoma
40
"strap cells" with eccentric nucleus with abundant granular eosinophilic cytoplasm with cross striations
rhabdomyosarcoma
41
desmin, myoglobin, MyoD1
rhabdomyosarcoma
42
strap cells
rhabdomyosarcoma
43
[...] rhabdomyosarcoma
site: head, neck, GU
epidemiology: children
clinical: rapidly growing mass
embryonal rhabdomyosarcoma
44
[...] rhabdomyosarcoma
site: head, neck, extremities
epidemiology: adolescents, all ages
clinical: rapidly growing mass
alveolar rhabdomyosarcoma
45
[...] rhabdomyosarcoma
site: extremities
epidemiology: older adults
clinical: rapidly growing mass
pleomorphic rhabdomyosarcoma
46
sarcoma botryoides "bunch of grapes"
embryonal rhabdomyosarcoma
47
most frequently occurs in children under 5 yo
develops in mucosal linings of nasopharynx, bile duct, vagina, bladder
embryonal rhabdomyosarcoma
48
characteristic t(2;13)(q35;14) translocation
PAX-OXO1 translocation
alveolar rhabdomyosarcoma
49
FISH detects FOXO1 breakpoint
alveolar rhabdomyosarcoma
50
PCR detects FOXO1-PAX fusion
alveolar rhabdomyosarcoma
51
in alveolar rhabdomyosarcoma, the chimeric PAX3-FOXO1 protein leads to dysregulation of
skeletal muscle differentiation
52
high grade small round cell tumor
solid growth pattern with central loss of cohesion
alveolar rhabdomyosarcoma
53
solid growth pattern
small round cells
loss of cohesion
alveolar rhabdomyosarcoma
54
list the prognosis of best to worst of the rhabdomyosarcomas
botryoid > embryonal > pleomorphic > alveolar
55
juvenille [...] of the skin of newborns is extremely common
capillary hemangiomas
56
closely packed aggregates of thin walled capillaries
capillary hemangioma
57
large cavernous vascular spaces filled with blood
often in liver or other deep structures
cavernous hemangiomas
58
associated with HHV8
kaposi sarcoma
59
most aggressive form of kaposi sarcoma
AIDS-associated
60
most common AIDS associated tumor in US
kaposi sarcoma
61
3 stages of kaposi sarcoma
patches
plaques
nodules
62
proliferation of slit-like vascular channels lined by plump spindle cells with extravasated RBCs
kaposi sarcoma
63
slit like spaces lined by spindle cells
kaposi sarcoma
64
associated with chemical carcinogens like arsenic, thorotrast, PVC
angiosarcoma
65
associated with lymphedema following axillary lymph node dissection in radical mastectomy for breast cancer
angiosarcoma
66
vascular channels lined by atypical plump malignant epithelial cells
angiosarcoma
67
CD31+
angiosarcoma
68
very aggressive vascular tumor with poor outcomes
frequent recurrence and distant metastasis
angiosarcoma
69
anastomosing vascular channel lined by atypical cells
angiosarcoma
70
occur in 20-40 yo
eccentric mass loosely attached to nerve
schwannoma
71
spindle cells show nuclear palisading, Antoni A and B areas
schwannoma
72
S100+ in **all** cells
schwannoma
73
small number associated with NF2
schwannoma
74
nuclear palisading (line up)
schwannoma
75
schwannoma are [encapsulated/nonencapulated] while neurofibromas are [encapsulated/nonencapulated]
schwannoma are encapuslated
neurofibromas are non-encapsulated
76
bundles of spindle cells with angulated, elongated nuclei
wavy cell process in loose mxyoid background
neurofibroma
77
S100+ in **scattered** cells
neurofibroma
78
10% occur in NF1
neurofibroma
79
non-encapsulated
mxyoid background
elongated spindle cells
neurofibroma
80
cafe-au-lait marks
elephantiasis
associated with
NF1 (neurofibroma)
81
plexiform neurofibroma is NF[..]
NF1
82
50% are associated with NF1
50% are sporadic
malignant peripheral nerve sheath tumor
83
70% of malignant peripheral nerve sheath tumor arise in
major nerve trunks
84
focal S100 in 60%
malignant peripheral nerve sheath tumor
85
spindle and epithelioid variants
malignant peripheral nerve sheath tumor
86
malignant peripheral nerve sheath tumor + rhabdomyosarcoma is called
Triton tumor
87
can arise from plexiform neurofibroma
malignant peripheral nerve sheath tumor
88
translocation t(x;18)
synovial sarcoma
89
biphasic tumor with epithelial and mesenchymal components
synovial sarcoma
90
commonly metastasizes to bone, lung, and lymph nodes
sometimes up to 20 years later
synovial sarcoma
91
mesenchymal (spindles) and epithelial elements
synovial sarcoma
92
malignant spindle cells in storiform pattern
large bizarre multinucleated pleomorphic cells
undifferentiated pleomorphic sarcoma
93
large, bizarre tumor cells
undifferentiated pleomorphic sarcoma
94
most commonly found in superficial subcutaneous tissue of the proximal extremities and trunk
lipoma
95
benign, soft, mobile, painless masses that are not removed except for cosmetic/functional purposes
lipoma
96
treatment for liposarcoma
wide excision
97
MDM2 amplification
well differentiated/dedifferentiated liposarcoma
98
fusion of DDIT3-FUS genes
t(12;16)
myxoid/round cell liposarcoma
99
mature fibroblasts surrounded by dense collagen
fibromatosis
100
types of fibromatosis [3]
penile
palmar
plantar
101
soft, mobile, subcutaneous mass
lipoma
102
large red plaques in mouth
Kaposi sarcoma
103
associated with mutation in beta catenin gene
deep seated fibromatosis
104
intraabdominal lesions are associated with familial adenomatous polyposis
deep seated fibromatosis
105
diagnostic cells of rhabdomyosarcoma
"strap cells"/rhabdomyoblasts
106
tumor that develops in hollow, mucosal lined organs
sarcoma botryoides (variant of embryonal rhabdomyosarcoma)
107
grossly resembles a bunch of grapes
sarcoma botryoides
108
common locations for sarcoma botryoides [4]
nasopharynx
bile duct
bladder
vagina
109
three types of rhabdomyosarcoma
embryonal
alveolar
pleomorphic
110
blunt ended, cigar shaped nuclei with minimal atypia and few mitoses
leiomyoma
111
commonly called uterine fibroids
leiomyoma
112
three types of hemangiomas
capillary
cavernous
pyogenic granuloma
113
most juvenille hemangiomas disappear by [...] of age
12 months
114
found in 95% percent of cases of Kaposi sarcoma
positive HHV-8 serology
115
common site for liposarcoma
retroperitoneum
116
often arise from perinephric fat
liposarcoma
117
what is the cell of origin of liposarcoma?
lipoblasts
118
what genetic factor is found in well differentiated/dedifferentiated liposarcoma?
MDM2 amplification
119
treatment of dedifferentiated liposarcoma
wide excision with possible chemotherapy/radiation as this is a high grade tumor with high potential of metastasis
120
when does mxyoid liposarcoma become high grade?
as the cells start to round out
121
cafe au lait spots are associated with
NF1
122
schwannomas are associated with NF[...]
NF2
123
neurofibromas are associated with NF[...]
NF1
124
grow diffusely within a nerve and resemble a "bag of worms"
plexiform neurofibroma (NF1)
125
50% of MPNST are associated with NF[..]
NF1
126
attached to nerve
malignant, grows rapidly
MPNST
**NF1 patients are high risk and need to be monitored closely for development of MPNST
127
can arise from the transformation of a neurofibroma, usually a plexiform
MPNST
128
slow-growing plaque like mass
slit like vascular spaces lined by spindle cells
extravasated RBCs
Kaposi sarcoma classic type
129
typically occurs in older men from middle east or eastern europe
slowly progressive
Kaposi sarcoma classic type
130
types of Kaposi sarcoma [4]
classic
African endemic
AIDS associated
transplant associated
131
classic translocation is t(2;13)
alveolar rhabdomyosarcoma
132
most common site for alveolar rhabdomyosarcoma
head/neck in adolescents
133
small round cell tumor with central discohesion
positive for skeletal muscle markers and rhabdomyoblasts
alveolar rhabdomyosarcoma
134
which rhabdomyosarcoma has the worst prognosis?
alveolar
135
biphasic with epithelial and mesenchymal markers
synovial sarcoma
136
most common site for synovial sarcoma
around knee
137
t(x;18) (p11;q11)
synovial sarcoma
138
SS18-SSX fusion on PCR
synovial sarcoma
139
SYT breakapart probe on FISH
synovial sarcoma
140
TLE-1 on IHC
synovial sarcoma
141
most common location of metastasis of synovial sarcoma
90% lungs
**10% to bones/lymph
142
metastasis occurs in 45% of [...] patients
synovial sarcoma
143
late metastasis can appear up to 20 years later
synovial sarcoma
144
CD31 and CD34 are markers of
vascular endothelial cells
145
cytokeratin is positive in [...] cells
epithelial
146
S100 is a marker of [...] differentiation
neural
147
MyoD is a marker of [...] differentiation
muscle
148
beta catenin
fibromatosis
149
DDIT3-FUS
round/myxoid liposarcoma
150
desmin is a marker of [...] differentiation
muscle
151
expression of smooth muscle actin indicates
leiomyoma
152
the presence of hemorrhage, necrosis, and frequent mitotic figures indicates
malignant tumor
153
alveolar rhabdomyosarcoma is associated with translocations involving
FOXO1
154
t(12;16)
round/myxoid liposarcoma
155
t(x;18)
synovial sarcoma
156
what type of tumors are CD31 and CD34 associated with?
vascular
157
