B6-016 Soft Tissue Sarcoma Flashcards

1
Q

what age group does rhabdomyosarcoma typically present in?

A

children

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2
Q

what age group does synovial sarcoma typically present in?

A

young adults

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3
Q

what age group does liposarcoma typically present in?

A

mid to late adult life

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4
Q

most common soft tissue tumors in adults

A

lipoma

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5
Q

encapsulated mass of mature fat cells

A

lipoma

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6
Q

one of the most common sarcomas in adults

A

liposarcoma

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7
Q

caused by amplification of the MDM2 gene

A

well differentiated/defifferentiated liposarcoma

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8
Q

chromosomal translocation t(12, 16)(q13;11)
fusion of DDIT3(CHOP) gene with FUS gene

A

mixoid/round cell liposarcoma

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9
Q

cytoplasmic vacuoles of lipid that scallop the nucleus

A

liposarcoma

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10
Q

lipoblasts and MDM2 amplification

A

well differentiated liposarcoma

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11
Q

t(12;16)(q13;11) tranlocation
DDIT3-FUS fusion

A

myxoid liposarcoma

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12
Q

translocation associated with myxoid/round cell liposarcoma

A

t(12;16)(q13;11)

DDIT3-FUS fusion

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13
Q

reactive pseudosarcomatous proliferation

A

nodular fasciitis

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14
Q

acute history of solitary rapidly growing mass often on volar aspect of forearm, chest, or back

A

nodular fasciitis

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15
Q

plump randomly arranged spindle cells in a myxoid background containing lymphocytes and extravasated RBCs

A

nodular fasciitis

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16
Q

fibroblasts
myxoid background
extravasated RBCs

A

nodular fasciitis

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17
Q

superficial fibromatosis occuring in the palm

A

dupuytren’s

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18
Q

superficial fibromatosis occuring in the plantar foot

A

ledderhosens

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19
Q

superficial fibromatosis occuring in the penis

A

peronie’s

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20
Q

nodular or poorly defined bundles of mature fibroblasts surrounded by dense collagen

A

superficial fibromatosis

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21
Q

collagen
fibroblasts

A

superficial fibromatosis

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22
Q

caused by mutation in the beta-catenin genes

A

deep seated fibromatosis

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23
Q

most frequently occur in females teens-30s

A

deep seated fibromatosis

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24
Q

often occur in patients with Gardner syndrome (variant of familial adenomatous polyposis)

A

intra-abdominal deep seated fibromatosis

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25
Q

grey white, firm infiltrative mass

A

deep seated fibromatosis

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26
Q

long fasicles of elongated fibroblasts and collagen fibers
IHC + for beta-catenin

A

deep seated fibromatosis

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27
Q

fibroblasts
trapped skeletal muscle

A

deep seated fibromatosis

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28
Q

rare sarcoma occuring mostly in the retroperitoneum, thigh, knee, or distal extremities

A

fibrosarcoma

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29
Q

malignant spindle cells arranged in herringbone pattern with frequent mitoses and areas of necrosis

A

fibrosarcoma

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30
Q

very aggressive fibrous tumor with high recurrence and metastasis rate

A

fibrosarcoma

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31
Q

herring bone pattern

A

fibrosarcoma

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32
Q

benign tumor of smooth muscle common in the uterus

A

leiomyoma

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33
Q

intersecting bundles of spindle cells with blunt-ended cigar shaped nuclei

A

leiomyoma

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34
Q

blunt ended cigar shaped nuclei, no atypia, no mitosis

A

leiomyoma

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35
Q

associated with smooth muscle actin (SMA), H-Caldesmon

A

leiomyosarcoma

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36
Q

malignant spindle cells with cigar shaped nuclei, frequent mitoses and necrosis

A

leiomyosarcoma

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37
Q

smooth muscle tumor with necrosis and hemorrhage

A

leiomyosarcoma

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38
Q

cigar shaped atypical nuclei

A

leiomyosarcoma

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39
Q

most common soft tissue sarcoma of children and adolescents

A

rhabdomyosarcoma

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40
Q

“strap cells” with eccentric nucleus with abundant granular eosinophilic cytoplasm with cross striations

A

rhabdomyosarcoma

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41
Q

desmin, myoglobin, MyoD1

A

rhabdomyosarcoma

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42
Q

strap cells

A

rhabdomyosarcoma

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43
Q

[…] rhabdomyosarcoma
site: head, neck, GU
epidemiology: children
clinical: rapidly growing mass

A

embryonal rhabdomyosarcoma

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44
Q

[…] rhabdomyosarcoma
site: head, neck, extremities
epidemiology: adolescents, all ages
clinical: rapidly growing mass

A

alveolar rhabdomyosarcoma

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45
Q

[…] rhabdomyosarcoma
site: extremities
epidemiology: older adults
clinical: rapidly growing mass

A

pleomorphic rhabdomyosarcoma

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46
Q

sarcoma botryoides “bunch of grapes”

A

embryonal rhabdomyosarcoma

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47
Q

most frequently occurs in children under 5 yo
develops in mucosal linings of nasopharynx, bile duct, vagina, bladder

A

embryonal rhabdomyosarcoma

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48
Q

characteristic t(2;13)(q35;14) translocation
PAX-OXO1 translocation

A

alveolar rhabdomyosarcoma

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49
Q

FISH detects FOXO1 breakpoint

A

alveolar rhabdomyosarcoma

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50
Q

PCR detects FOXO1-PAX fusion

A

alveolar rhabdomyosarcoma

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51
Q

in alveolar rhabdomyosarcoma, the chimeric PAX3-FOXO1 protein leads to dysregulation of

A

skeletal muscle differentiation

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52
Q

high grade small round cell tumor
solid growth pattern with central loss of cohesion

A

alveolar rhabdomyosarcoma

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53
Q

solid growth pattern
small round cells
loss of cohesion

A

alveolar rhabdomyosarcoma

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54
Q

list the prognosis of best to worst of the rhabdomyosarcomas

A

botryoid > embryonal > pleomorphic > alveolar

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55
Q

juvenille […] of the skin of newborns is extremely common

A

capillary hemangiomas

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56
Q

closely packed aggregates of thin walled capillaries

A

capillary hemangioma

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57
Q

large cavernous vascular spaces filled with blood
often in liver or other deep structures

A

cavernous hemangiomas

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58
Q

associated with HHV8

A

kaposi sarcoma

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59
Q

most aggressive form of kaposi sarcoma

A

AIDS-associated

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60
Q

most common AIDS associated tumor in US

A

kaposi sarcoma

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61
Q

3 stages of kaposi sarcoma

A

patches
plaques
nodules

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62
Q

proliferation of slit-like vascular channels lined by plump spindle cells with extravasated RBCs

A

kaposi sarcoma

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63
Q

slit like spaces lined by spindle cells

A

kaposi sarcoma

64
Q

associated with chemical carcinogens like arsenic, thorotrast, PVC

A

angiosarcoma

65
Q

associated with lymphedema following axillary lymph node dissection in radical mastectomy for breast cancer

A

angiosarcoma

66
Q

vascular channels lined by atypical plump malignant epithelial cells

A

angiosarcoma

67
Q

CD31+

A

angiosarcoma

68
Q

very aggressive vascular tumor with poor outcomes
frequent recurrence and distant metastasis

A

angiosarcoma

69
Q

anastomosing vascular channel lined by atypical cells

A

angiosarcoma

70
Q

occur in 20-40 yo
eccentric mass loosely attached to nerve

A

schwannoma

71
Q

spindle cells show nuclear palisading, Antoni A and B areas

A

schwannoma

72
Q

S100+ in all cells

A

schwannoma

73
Q

small number associated with NF2

A

schwannoma

74
Q

nuclear palisading (line up)

A

schwannoma

75
Q

schwannoma are [encapsulated/nonencapulated] while neurofibromas are [encapsulated/nonencapulated]

A

schwannoma are encapuslated
neurofibromas are non-encapsulated

76
Q

bundles of spindle cells with angulated, elongated nuclei
wavy cell process in loose mxyoid background

A

neurofibroma

77
Q

S100+ in scattered cells

A

neurofibroma

78
Q

10% occur in NF1

A

neurofibroma

79
Q

non-encapsulated
mxyoid background
elongated spindle cells

A

neurofibroma

80
Q

cafe-au-lait marks
elephantiasis
associated with

A

NF1 (neurofibroma)

81
Q

plexiform neurofibroma is NF[..]

A

NF1

82
Q

50% are associated with NF1
50% are sporadic

A

malignant peripheral nerve sheath tumor

83
Q

70% of malignant peripheral nerve sheath tumor arise in

A

major nerve trunks

84
Q

focal S100 in 60%

A

malignant peripheral nerve sheath tumor

85
Q

spindle and epithelioid variants

A

malignant peripheral nerve sheath tumor

86
Q

malignant peripheral nerve sheath tumor + rhabdomyosarcoma is called

A

Triton tumor

87
Q

can arise from plexiform neurofibroma

A

malignant peripheral nerve sheath tumor

88
Q

translocation t(x;18)

A

synovial sarcoma

89
Q

biphasic tumor with epithelial and mesenchymal components

A

synovial sarcoma

90
Q

commonly metastasizes to bone, lung, and lymph nodes
sometimes up to 20 years later

A

synovial sarcoma

91
Q

mesenchymal (spindles) and epithelial elements

A

synovial sarcoma

92
Q

malignant spindle cells in storiform pattern
large bizarre multinucleated pleomorphic cells

A

undifferentiated pleomorphic sarcoma

93
Q

large, bizarre tumor cells

A

undifferentiated pleomorphic sarcoma

94
Q

most commonly found in superficial subcutaneous tissue of the proximal extremities and trunk

A

lipoma

95
Q

benign, soft, mobile, painless masses that are not removed except for cosmetic/functional purposes

A

lipoma

96
Q

treatment for liposarcoma

A

wide excision

97
Q

MDM2 amplification

A

well differentiated/dedifferentiated liposarcoma

98
Q

fusion of DDIT3-FUS genes
t(12;16)

A

myxoid/round cell liposarcoma

99
Q

mature fibroblasts surrounded by dense collagen

A

fibromatosis

100
Q

types of fibromatosis [3]

A

penile
palmar
plantar

101
Q

soft, mobile, subcutaneous mass

A

lipoma

102
Q

large red plaques in mouth

A

Kaposi sarcoma

103
Q

associated with mutation in beta catenin gene

A

deep seated fibromatosis

104
Q

intraabdominal lesions are associated with familial adenomatous polyposis

A

deep seated fibromatosis

105
Q

diagnostic cells of rhabdomyosarcoma

A

“strap cells”/rhabdomyoblasts

106
Q

tumor that develops in hollow, mucosal lined organs

A

sarcoma botryoides (variant of embryonal rhabdomyosarcoma)

107
Q

grossly resembles a bunch of grapes

A

sarcoma botryoides

108
Q

common locations for sarcoma botryoides [4]

A

nasopharynx
bile duct
bladder
vagina

109
Q

three types of rhabdomyosarcoma

A

embryonal
alveolar
pleomorphic

110
Q

blunt ended, cigar shaped nuclei with minimal atypia and few mitoses

A

leiomyoma

111
Q

commonly called uterine fibroids

A

leiomyoma

112
Q

three types of hemangiomas

A

capillary
cavernous
pyogenic granuloma

113
Q

most juvenille hemangiomas disappear by […] of age

A

12 months

114
Q

found in 95% percent of cases of Kaposi sarcoma

A

positive HHV-8 serology

115
Q

common site for liposarcoma

A

retroperitoneum

116
Q

often arise from perinephric fat

A

liposarcoma

117
Q

what is the cell of origin of liposarcoma?

A

lipoblasts

118
Q

what genetic factor is found in well differentiated/dedifferentiated liposarcoma?

A

MDM2 amplification

119
Q

treatment of dedifferentiated liposarcoma

A

wide excision with possible chemotherapy/radiation as this is a high grade tumor with high potential of metastasis

120
Q

when does mxyoid liposarcoma become high grade?

A

as the cells start to round out

121
Q

cafe au lait spots are associated with

A

NF1

122
Q

schwannomas are associated with NF[…]

A

NF2

123
Q

neurofibromas are associated with NF[…]

A

NF1

124
Q

grow diffusely within a nerve and resemble a “bag of worms”

A

plexiform neurofibroma (NF1)

125
Q

50% of MPNST are associated with NF[..]

A

NF1

126
Q

attached to nerve
malignant, grows rapidly

A

MPNST

**NF1 patients are high risk and need to be monitored closely for development of MPNST

127
Q

can arise from the transformation of a neurofibroma, usually a plexiform

A

MPNST

128
Q

slow-growing plaque like mass
slit like vascular spaces lined by spindle cells
extravasated RBCs

A

Kaposi sarcoma classic type

129
Q

typically occurs in older men from middle east or eastern europe
slowly progressive

A

Kaposi sarcoma classic type

130
Q

types of Kaposi sarcoma [4]

A

classic
African endemic
AIDS associated
transplant associated

131
Q

classic translocation is t(2;13)

A

alveolar rhabdomyosarcoma

132
Q

most common site for alveolar rhabdomyosarcoma

A

head/neck in adolescents

133
Q

small round cell tumor with central discohesion
positive for skeletal muscle markers and rhabdomyoblasts

A

alveolar rhabdomyosarcoma

134
Q

which rhabdomyosarcoma has the worst prognosis?

A

alveolar

135
Q

biphasic with epithelial and mesenchymal markers

A

synovial sarcoma

136
Q

most common site for synovial sarcoma

A

around knee

137
Q

t(x;18) (p11;q11)

A

synovial sarcoma

138
Q

SS18-SSX fusion on PCR

A

synovial sarcoma

139
Q

SYT breakapart probe on FISH

A

synovial sarcoma

140
Q

TLE-1 on IHC

A

synovial sarcoma

141
Q

most common location of metastasis of synovial sarcoma

A

90% lungs

**10% to bones/lymph

142
Q

metastasis occurs in 45% of […] patients

A

synovial sarcoma

143
Q

late metastasis can appear up to 20 years later

A

synovial sarcoma

144
Q

CD31 and CD34 are markers of

A

vascular endothelial cells

145
Q

cytokeratin is positive in […] cells

A

epithelial

146
Q

S100 is a marker of […] differentiation

A

neural

147
Q

MyoD is a marker of […] differentiation

A

muscle

148
Q

beta catenin

A

fibromatosis

149
Q

DDIT3-FUS

A

round/myxoid liposarcoma

150
Q

desmin is a marker of […] differentiation

A

muscle

151
Q

expression of smooth muscle actin indicates

A

leiomyoma

152
Q

the presence of hemorrhage, necrosis, and frequent mitotic figures indicates

A

malignant tumor

153
Q

alveolar rhabdomyosarcoma is associated with translocations involving

A

FOXO1

154
Q

t(12;16)

A

round/myxoid liposarcoma

155
Q

t(x;18)

A

synovial sarcoma

156
Q

what type of tumors are CD31 and CD34 associated with?

A

vascular

157
Q
A