SOFT TISSUE TUMORS Flashcards
(138 cards)
1
Q
why do soft tissue tumors develop
A
mutation in mesenchymal stem cells
2
Q
what type of chromosome abnormality is usually seen
A
translocation
3
Q
what does the translocation result in?
A
fusion gene that leads to chimeric transcription protein leading to uncontrolled cell proliferation
4
Q
factors used to classify tumor
A
histologic grade
5
Q
what tumors have a better prognosis
A
tumors arising in superficial location
6
Q
what does larger size correlate with
A
great chance of metastatic disease
7
Q
what studies are used to assess soft tissue tumors
A
MRI
8
Q
treatment normally
A
surgery (may have radiation therapy and chemotherapy in certain situations)
9
Q
what procedure is done for larger tumors
A
needle biopsy
10
Q
most common soft tissue tumor
A
lipoma
11
Q
lipoma
A
fatty tumor
12
Q
when do you uusually see lipomas
A
young adults or older (not first two decades of life)
13
Q
do lipomas metastasize?
A
no
14
Q
where do lipomas occur
A
superficial locations
15
Q
most common lipoma with mature fat only
A
conventional lipoma
16
Q
mature fat plus small vessel proliferation, can be painful
A
angiolipoma
17
Q
mature fat plus spindle cells
A
spindle cell lipoma
18
Q
mature fat plus pleomorphic cells
A
pleomorphic lipoma
19
Q
lipoma that involves skeletal muscle, not well circumscribed
A
intramuscular lipoma
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
21
Q
most common tumor of adulthood
A
liposarcoma
22
Q
what age to liposarcomas occur
A
middle to older age adults
23
Q
where do liposarcomas arise
A
deep soft tissue of proximal extremities and retroperitoneum
24
Q
preferred imaging modality for soft tissue tumors
A
mri
25
indolent, local recurrence, can be fatal
well differentiated liposarcoma
26
intermediate malignancy
myxoid/round liposarcoma
27
aggressive and frequently metastasizes
pleomorphic liposarcoma
28
non neoplastic bening lesions that develop following local trauma or are idiopathic
reactive pseudosarcomatous proliferations
29
2 types of reactive pseudosarcomatous proliferations
nodular fasciitis
30
occurs in upper extremities at any age
nodular fascitis
31
usually develops in atheletic adolescents and young adults often following epsiode of trauma, has reactive proliferative lesions
myositis ossificans
32
has rapidly growing painful mass (only had for a couple weeks)
nodular fasciitis
33
found in muscles of proximal extremities and overtime the outer most layer becomes ossified
myositis ossificans
34
characteristics of superficial fibromatoses
involve fascia or broad tendons
35
do superficial firbomatoses metastasize?
no they're benign
36
type that can cause skin puckering and finger can't be extended
palmar fibromatosis (dupuytren's contracture)
37
2 other types of superficial fibromatoses
plantar fibromatosis
38
benign tumors that can occur at any age. Show infiltrative poorly marginated proliferations in fibroblasts with collagen
deep seated fibromatosis
39
what do almost all patients with deep seated fibromatosis have
somatic beta catenin or adenomatous polyposis coli mutations (APC gene) that could lead to colon cancer
40
3 main types of deep seated fibromatosis
abdominal
41
malignant tumor of fibroblasts
fibrosarcoma
42
where are fibrosarcomas common
deep tissue of the extremities
43
prognosis of fibrosarcomas
very aggressive
44
fibrous tumor that occurs in infants and young children
fibromatosis coli
45
what causes fibromatosis coli
cellular scar reaction to injury of the sternoclediomastoid muscle
46
contain cellular elements that resemble fibroblasts and histocytes
fibrohistiocytic tumor
47
intermediate malignancy, prone to local recurrences
dermatofibrosarcoma protuberans (dermatofibroma)
48
once most common sarcoma in adults - was reclassified
malignant fibrous histiocytoma (NFH)
49
new classification of NFH
undifferentiated pleomorphic sarcoma
50
where does NFH occur
older adults (men) in deep tissues of thigh followed up upper exremities
51
behavior of NFH
similar to high grade sarcomas
52
benign tumor of skeletal muscle
rhabodmyoma
53
very rare, may occur in heart
rhabdomyoma
54
children rhabdomyoma
cardiac w/tuberous sclerosis
55
adult rhabdomyoma
head, neck, or genital region
56
most common soft tissue sarcoma of childhood or adolescence, very aggressive
rhadomyosarcoma
57
treatment for rhadomyosarcoma
multimodal therapy
58
most common subtype of rhadomyosarcoma
embryonal rhadomyosarcoma
59
when does embryonal rhadomyosarcoma occur
in children age 10 or younger
60
where does embryonal rhadomyosarcoma occur
head or neck or genitourinary tract
61
subtype that arises in the mucosal lining of hollow organs and forms polypoid, grape like growth, has the best chance of survival
botryoid subtype
62
occurs in early to middle adolescence
alveolar rhadomyosarcoma
63
where does alveolar rhadomyosarcoma occur
deep soft tissue of extremities
64
what is alveolar rhadomyosarcoma based on
certain fusion genes
65
rarest type of rhadomyosarcoma that is in the deep soft tissue of adults
pleomorphic rhadomyosarcoma
66
2 tumors of smooth muscle
leiomyoma
67
where do most leiomyomas arise
the uterus
68
what are leiomyomas called
uterine fibroids (present in women)
69
what can leiomyomas arise from
erector pili muscles of skin, nipples, scrotum, labia
70
are leiomyomas malignant
no
71
malignant type of smooth muscle tumor
leiomysarcoma
72
where do leiomyosarcomas occur most frequently
uterus or GI tract
73
benign tumor of blood vessel that occurs at ANY SITE ON SKIN
hemangioma
74
benign tumor of blood vessel that is eruptive
pyogenic
75
benign tumor of blood vessel in skin and subcutaneous of soft tissue. may occur in neck or axilla of children
lymphangioma
76
what could cystic hygroma of neck in children be associated with
turner syndrome
77
benign tumor of blood vessel that is painful and shows modification of glomus body. Occurs in DISTAL DIGITS
glomus tumor and glomangioma
78
not true neoplasms, dilations of prexisitng vessels
vascular ectasia
79
usually on head or neck of infants
nevus flammeus (birthmark)
80
spider like seen w/excess estrogen
spider angiomas
81
autosomal dominant disorder w/vascular ectasias over skin, oral mucous membranes, respiratory, GI
hereditary hemorrhagic telangiectasia
82
benign tumor of blood vessel that is reactive vascular proliferation resulting from OPPORTUNISTIC INFECTION
bacillary angiomatosis
83
what causes bacillary angiomatosis
infection w/bartonella bacteria in immunocompromsied
84
where do bacillary angiomatosis lesions occur
anywhere
85
treatment for bacillary angiomatosis
antibiotics
86
malignant tumor of blood vessels that is aggressive
angiosarcoma
87
who is affected by angiosarcomas
older adults
88
presentation of angiosarcomas
skin of head and neck, scalp, soft tissue, breast, liver
89
what could angiosarcoma be secondary to
radiation therapy
90
what is seen with angiosarcomas
dilated lymphatics
91
less aggressive malignant blood vessel tumor and is very rare
hemangioendothelioma
92
infectious sarcoma
kaposis sarcoma
93
cause of kaposis sarcoma
HHV 8
94
virus in concert w/cofactor leads to...
endothelial cell proliferation
95
4 types of kaposi sarcoma
AIDS asscoiated
96
2 peripheral nerve tumor-like lesions
Morton neuroma
97
caused by repeated minor trauma, typically occurs in planter nerve between third and fourth toes
morton neuroma
98
occurs when peripheral nerve is severed or crushed
traumatic neuroma
99
3 types of peripheral nerve sheath tumors
schwannoma
100
arises from schwann cell and is benign
schwannoma
101
what may schwannoma be associated with?
neurofibromatosis type 2 (NF2)
102
benign tumor in discrete localized mass
neurofibroma
103
what does presence of multiple neurofibromas or plexiform neurofibromas result in or suggest?
diagnosis of neurofibromatosis type I
104
type of disorder of neurofibromtosis
autosomal domiant
105
what do the mutations act as
growth suppressors
106
what type is more common
type I
107
type I mutation
codes for neurofibromin on chromosome 17
108
peripheral type
type I
109
what is seen with type I neurofibromatosis
cafe au late spots
110
type II mutation
codes for merlin
111
what does merlin do
regulates membrane receptor signaling on chromosome 22
112
central type
type 2
113
what is type 2 associated with
bilateral acoustic schwannomas
114
where do malignant peripheral nerve sheath tumors occur
nerve trunks or proximal portions of upper and lower extremities or trunk
115
what do 50% of MPNSTs occur with
NF1
116
highly malignant sarcoma
MPNST
117
lesions of synovial origin
tenosynovial giant cell tumor
118
benign tumor either localized or diffuse characterized by fusion gene abnormality
tenosynovial giant cell tumor
119
presents as solitary and slow growing, involves tendon sheaths of fingers and wrists
localized form of tenosynovial giant cell tumor
120
most common mesenchymal neoplasm of hand
localized tenosynovial giant cell tumor
121
presents in knee followed by ankle and foot, rare
diffuse form of tenosynovial giant cell tumor
122
doesn't show synovial cell differentiation and is uncommon in joint cavity
synovial sarcoma
123
where does synovial sarcoma present
para articular regions of extremites, less common in head and neck
124
age group affected w/synovial sarcoma
adolescents and young adults
125
epithelial and spindle cell components
biphasic type of synovial sarcoma
126
spindle cell component only of synovial sarcoma
monophasic type
127
what test is helpful in diagnosis of synovial sarcoma
immunohistochemistry
128
what is immunohistochemistry postive for with synovial sarcoma
cytokeratin (epithelial marker)
129
abnormal fusion in synovial sarcoma
SYT-SSX
130
extracellular MUCIN accumulation assoicated with cellular proliferation
myxomatous lesions
131
examples of myxomatous lesions
intramusclar myxoma
132
common myxomatous lesion that isn't a neoplasm, occurs in fibrous connective tissue almost always near joint capsule or tendon sheath
ganglion cyst
133
most common location of myxomatous lesion
wrist
134
what are lesions known as on the fingers
digital mucous cysts
135
most common mesenchymal lesion of the hand and wrist
ganglion and digital mucus cyst
136
common benign lesions
lipomas
137
common adult sarcomas
undifferentiated pleomorphic sarcoma
138
common childhood sarcoma
rhabdomyosarcoma
