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SKIN/MS FINAL > SOFT TISSUE TUMORS > Flashcards

SOFT TISSUE TUMORS Flashcards

1
Q

why do soft tissue tumors develop

A

mutation in mesenchymal stem cells

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2
Q

what type of chromosome abnormality is usually seen

A

translocation

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3
Q

what does the translocation result in?

A

fusion gene that leads to chimeric transcription protein leading to uncontrolled cell proliferation

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4
Q

factors used to classify tumor

A

histologic grade

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5
Q

what tumors have a better prognosis

A

tumors arising in superficial location

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6
Q

what does larger size correlate with

A

great chance of metastatic disease

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7
Q

what studies are used to assess soft tissue tumors

A

MRI

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8
Q

treatment normally

A

surgery (may have radiation therapy and chemotherapy in certain situations)

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9
Q

what procedure is done for larger tumors

A

needle biopsy

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10
Q

most common soft tissue tumor

A

lipoma

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11
Q

lipoma

A

fatty tumor

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12
Q

when do you uusually see lipomas

A

young adults or older (not first two decades of life)

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13
Q

do lipomas metastasize?

A

no

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14
Q

where do lipomas occur

A

superficial locations

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15
Q

most common lipoma with mature fat only

A

conventional lipoma

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16
Q

mature fat plus small vessel proliferation, can be painful

A

angiolipoma

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17
Q

mature fat plus spindle cells

A

spindle cell lipoma

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18
Q

mature fat plus pleomorphic cells

A

pleomorphic lipoma

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19
Q

lipoma that involves skeletal muscle, not well circumscribed

A

intramuscular lipoma

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20
Q

who could get overgrowth of mature adipose tissue in face or back (buffalo hump)

A

patients on long term corticosteroids - called steroid lipomatosis

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21
Q

most common tumor of adulthood

A

liposarcoma

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22
Q

what age to liposarcomas occur

A

middle to older age adults

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23
Q

where do liposarcomas arise

A

deep soft tissue of proximal extremities and retroperitoneum

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24
Q

preferred imaging modality for soft tissue tumors

A

mri

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25
Q

indolent, local recurrence, can be fatal

A

well differentiated liposarcoma

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26
Q

intermediate malignancy

A

myxoid/round liposarcoma

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27
Q

aggressive and frequently metastasizes

A

pleomorphic liposarcoma

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28
Q

non neoplastic bening lesions that develop following local trauma or are idiopathic

A

reactive pseudosarcomatous proliferations

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29
Q

2 types of reactive pseudosarcomatous proliferations

A

nodular fasciitis

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30
Q

occurs in upper extremities at any age

A

nodular fascitis

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31
Q

usually develops in atheletic adolescents and young adults often following epsiode of trauma, has reactive proliferative lesions

A

myositis ossificans

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32
Q

has rapidly growing painful mass (only had for a couple weeks)

A

nodular fasciitis

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33
Q

found in muscles of proximal extremities and overtime the outer most layer becomes ossified

A

myositis ossificans

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34
Q

characteristics of superficial fibromatoses

A

involve fascia or broad tendons

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35
Q

do superficial firbomatoses metastasize?

A

no they’re benign

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36
Q

type that can cause skin puckering and finger can’t be extended

A

palmar fibromatosis (dupuytren’s contracture)

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37
Q

2 other types of superficial fibromatoses

A

plantar fibromatosis

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38
Q

benign tumors that can occur at any age. Show infiltrative poorly marginated proliferations in fibroblasts with collagen

A

deep seated fibromatosis

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39
Q

what do almost all patients with deep seated fibromatosis have

A

somatic beta catenin or adenomatous polyposis coli mutations (APC gene) that could lead to colon cancer

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40
Q

3 main types of deep seated fibromatosis

A

abdominal

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41
Q

malignant tumor of fibroblasts

A

fibrosarcoma

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42
Q

where are fibrosarcomas common

A

deep tissue of the extremities

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43
Q

prognosis of fibrosarcomas

A

very aggressive

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44
Q

fibrous tumor that occurs in infants and young children

A

fibromatosis coli

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45
Q

what causes fibromatosis coli

A

cellular scar reaction to injury of the sternoclediomastoid muscle

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46
Q

contain cellular elements that resemble fibroblasts and histocytes

A

fibrohistiocytic tumor

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47
Q

intermediate malignancy, prone to local recurrences

A

dermatofibrosarcoma protuberans (dermatofibroma)

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48
Q

once most common sarcoma in adults - was reclassified

A

malignant fibrous histiocytoma (NFH)

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49
Q

new classification of NFH

A

undifferentiated pleomorphic sarcoma

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50
Q

where does NFH occur

A

older adults (men) in deep tissues of thigh followed up upper exremities

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51
Q

behavior of NFH

A

similar to high grade sarcomas

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52
Q

benign tumor of skeletal muscle

A

rhabodmyoma

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53
Q

very rare, may occur in heart

A

rhabdomyoma

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54
Q

children rhabdomyoma

A

cardiac w/tuberous sclerosis

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55
Q

adult rhabdomyoma

A

head, neck, or genital region

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56
Q

most common soft tissue sarcoma of childhood or adolescence, very aggressive

A

rhadomyosarcoma

57
Q

treatment for rhadomyosarcoma

A

multimodal therapy

58
Q

most common subtype of rhadomyosarcoma

A

embryonal rhadomyosarcoma

59
Q

when does embryonal rhadomyosarcoma occur

A

in children age 10 or younger

60
Q

where does embryonal rhadomyosarcoma occur

A

head or neck or genitourinary tract

61
Q

subtype that arises in the mucosal lining of hollow organs and forms polypoid, grape like growth, has the best chance of survival

A

botryoid subtype

62
Q

occurs in early to middle adolescence

A

alveolar rhadomyosarcoma

63
Q

where does alveolar rhadomyosarcoma occur

A

deep soft tissue of extremities

64
Q

what is alveolar rhadomyosarcoma based on

A

certain fusion genes

65
Q

rarest type of rhadomyosarcoma that is in the deep soft tissue of adults

A

pleomorphic rhadomyosarcoma

66
Q

2 tumors of smooth muscle

A

leiomyoma

67
Q

where do most leiomyomas arise

A

the uterus

68
Q

what are leiomyomas called

A

uterine fibroids (present in women)

69
Q

what can leiomyomas arise from

A

erector pili muscles of skin, nipples, scrotum, labia

70
Q

are leiomyomas malignant

A

no

71
Q

malignant type of smooth muscle tumor

A

leiomysarcoma

72
Q

where do leiomyosarcomas occur most frequently

A

uterus or GI tract

73
Q

benign tumor of blood vessel that occurs at ANY SITE ON SKIN

A

hemangioma

74
Q

benign tumor of blood vessel that is eruptive

A

pyogenic

75
Q

benign tumor of blood vessel in skin and subcutaneous of soft tissue. may occur in neck or axilla of children

A

lymphangioma

76
Q

what could cystic hygroma of neck in children be associated with

A

turner syndrome

77
Q

benign tumor of blood vessel that is painful and shows modification of glomus body. Occurs in DISTAL DIGITS

A

glomus tumor and glomangioma

78
Q

not true neoplasms, dilations of prexisitng vessels

A

vascular ectasia

79
Q

usually on head or neck of infants

A

nevus flammeus (birthmark)

80
Q

spider like seen w/excess estrogen

A

spider angiomas

81
Q

autosomal dominant disorder w/vascular ectasias over skin, oral mucous membranes, respiratory, GI

A

hereditary hemorrhagic telangiectasia

82
Q

benign tumor of blood vessel that is reactive vascular proliferation resulting from OPPORTUNISTIC INFECTION

A

bacillary angiomatosis

83
Q

what causes bacillary angiomatosis

A

infection w/bartonella bacteria in immunocompromsied

84
Q

where do bacillary angiomatosis lesions occur

A

anywhere

85
Q

treatment for bacillary angiomatosis

A

antibiotics

86
Q

malignant tumor of blood vessels that is aggressive

A

angiosarcoma

87
Q

who is affected by angiosarcomas

A

older adults

88
Q

presentation of angiosarcomas

A

skin of head and neck, scalp, soft tissue, breast, liver

89
Q

what could angiosarcoma be secondary to

A

radiation therapy

90
Q

what is seen with angiosarcomas

A

dilated lymphatics

91
Q

less aggressive malignant blood vessel tumor and is very rare

A

hemangioendothelioma

92
Q

infectious sarcoma

A

kaposis sarcoma

93
Q

cause of kaposis sarcoma

A

HHV 8

94
Q

virus in concert w/cofactor leads to…

A

endothelial cell proliferation

95
Q

4 types of kaposi sarcoma

A

AIDS asscoiated

96
Q

2 peripheral nerve tumor-like lesions

A

Morton neuroma

97
Q

caused by repeated minor trauma, typically occurs in planter nerve between third and fourth toes

A

morton neuroma

98
Q

occurs when peripheral nerve is severed or crushed

A

traumatic neuroma

99
Q

3 types of peripheral nerve sheath tumors

A

schwannoma

100
Q

arises from schwann cell and is benign

A

schwannoma

101
Q

what may schwannoma be associated with?

A

neurofibromatosis type 2 (NF2)

102
Q

benign tumor in discrete localized mass

A

neurofibroma

103
Q

what does presence of multiple neurofibromas or plexiform neurofibromas result in or suggest?

A

diagnosis of neurofibromatosis type I

104
Q

type of disorder of neurofibromtosis

A

autosomal domiant

105
Q

what do the mutations act as

A

growth suppressors

106
Q

what type is more common

A

type I

107
Q

type I mutation

A

codes for neurofibromin on chromosome 17

108
Q

peripheral type

A

type I

109
Q

what is seen with type I neurofibromatosis

A

cafe au late spots

110
Q

type II mutation

A

codes for merlin

111
Q

what does merlin do

A

regulates membrane receptor signaling on chromosome 22

112
Q

central type

A

type 2

113
Q

what is type 2 associated with

A

bilateral acoustic schwannomas

114
Q

where do malignant peripheral nerve sheath tumors occur

A

nerve trunks or proximal portions of upper and lower extremities or trunk

115
Q

what do 50% of MPNSTs occur with

A

NF1

116
Q

highly malignant sarcoma

A

MPNST

117
Q

lesions of synovial origin

A

tenosynovial giant cell tumor

118
Q

benign tumor either localized or diffuse characterized by fusion gene abnormality

A

tenosynovial giant cell tumor

119
Q

presents as solitary and slow growing, involves tendon sheaths of fingers and wrists

A

localized form of tenosynovial giant cell tumor

120
Q

most common mesenchymal neoplasm of hand

A

localized tenosynovial giant cell tumor

121
Q

presents in knee followed by ankle and foot, rare

A

diffuse form of tenosynovial giant cell tumor

122
Q

doesn’t show synovial cell differentiation and is uncommon in joint cavity

A

synovial sarcoma

123
Q

where does synovial sarcoma present

A

para articular regions of extremites, less common in head and neck

124
Q

age group affected w/synovial sarcoma

A

adolescents and young adults

125
Q

epithelial and spindle cell components

A

biphasic type of synovial sarcoma

126
Q

spindle cell component only of synovial sarcoma

A

monophasic type

127
Q

what test is helpful in diagnosis of synovial sarcoma

A

immunohistochemistry

128
Q

what is immunohistochemistry postive for with synovial sarcoma

A

cytokeratin (epithelial marker)

129
Q

abnormal fusion in synovial sarcoma

A

SYT-SSX

130
Q

extracellular MUCIN accumulation assoicated with cellular proliferation

A

myxomatous lesions

131
Q

examples of myxomatous lesions

A

intramusclar myxoma

132
Q

common myxomatous lesion that isn’t a neoplasm, occurs in fibrous connective tissue almost always near joint capsule or tendon sheath

A

ganglion cyst

133
Q

most common location of myxomatous lesion

A

wrist

134
Q

what are lesions known as on the fingers

A

digital mucous cysts

135
Q

most common mesenchymal lesion of the hand and wrist

A

ganglion and digital mucus cyst

136
Q

common benign lesions

A

lipomas

137
Q

common adult sarcomas

A

undifferentiated pleomorphic sarcoma

138
Q

common childhood sarcoma

A

rhabdomyosarcoma

SKIN/MS FINAL (6 decks)

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