SOFT TISSUE TUMORS Flashcards
why do soft tissue tumors develop
mutation in mesenchymal stem cells
what type of chromosome abnormality is usually seen
translocation
what does the translocation result in?
fusion gene that leads to chimeric transcription protein leading to uncontrolled cell proliferation
factors used to classify tumor
histologic grade
what tumors have a better prognosis
tumors arising in superficial location
what does larger size correlate with
great chance of metastatic disease
what studies are used to assess soft tissue tumors
MRI
treatment normally
surgery (may have radiation therapy and chemotherapy in certain situations)
what procedure is done for larger tumors
needle biopsy
most common soft tissue tumor
lipoma
lipoma
fatty tumor
when do you uusually see lipomas
young adults or older (not first two decades of life)
do lipomas metastasize?
no
where do lipomas occur
superficial locations
most common lipoma with mature fat only
conventional lipoma
mature fat plus small vessel proliferation, can be painful
angiolipoma
mature fat plus spindle cells
spindle cell lipoma
mature fat plus pleomorphic cells
pleomorphic lipoma
lipoma that involves skeletal muscle, not well circumscribed
intramuscular lipoma
who could get overgrowth of mature adipose tissue in face or back (buffalo hump)
patients on long term corticosteroids - called steroid lipomatosis
most common tumor of adulthood
liposarcoma
what age to liposarcomas occur
middle to older age adults
where do liposarcomas arise
deep soft tissue of proximal extremities and retroperitoneum
preferred imaging modality for soft tissue tumors
mri
indolent, local recurrence, can be fatal
well differentiated liposarcoma
intermediate malignancy
myxoid/round liposarcoma
aggressive and frequently metastasizes
pleomorphic liposarcoma
non neoplastic bening lesions that develop following local trauma or are idiopathic
reactive pseudosarcomatous proliferations
2 types of reactive pseudosarcomatous proliferations
nodular fasciitis
occurs in upper extremities at any age
nodular fascitis
usually develops in atheletic adolescents and young adults often following epsiode of trauma, has reactive proliferative lesions
myositis ossificans
has rapidly growing painful mass (only had for a couple weeks)
nodular fasciitis
found in muscles of proximal extremities and overtime the outer most layer becomes ossified
myositis ossificans
characteristics of superficial fibromatoses
involve fascia or broad tendons
do superficial firbomatoses metastasize?
no they’re benign
type that can cause skin puckering and finger can’t be extended
palmar fibromatosis (dupuytren’s contracture)
2 other types of superficial fibromatoses
plantar fibromatosis
benign tumors that can occur at any age. Show infiltrative poorly marginated proliferations in fibroblasts with collagen
deep seated fibromatosis
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
3 main types of deep seated fibromatosis
abdominal
malignant tumor of fibroblasts
fibrosarcoma
where are fibrosarcomas common
deep tissue of the extremities
prognosis of fibrosarcomas
very aggressive
fibrous tumor that occurs in infants and young children
fibromatosis coli
what causes fibromatosis coli
cellular scar reaction to injury of the sternoclediomastoid muscle
contain cellular elements that resemble fibroblasts and histocytes
fibrohistiocytic tumor
intermediate malignancy, prone to local recurrences
dermatofibrosarcoma protuberans (dermatofibroma)
once most common sarcoma in adults - was reclassified
malignant fibrous histiocytoma (NFH)
new classification of NFH
undifferentiated pleomorphic sarcoma
where does NFH occur
older adults (men) in deep tissues of thigh followed up upper exremities
behavior of NFH
similar to high grade sarcomas
benign tumor of skeletal muscle
rhabodmyoma
very rare, may occur in heart
rhabdomyoma
children rhabdomyoma
cardiac w/tuberous sclerosis
adult rhabdomyoma
head, neck, or genital region
most common soft tissue sarcoma of childhood or adolescence, very aggressive
rhadomyosarcoma
treatment for rhadomyosarcoma
multimodal therapy
most common subtype of rhadomyosarcoma
embryonal rhadomyosarcoma
when does embryonal rhadomyosarcoma occur
in children age 10 or younger
where does embryonal rhadomyosarcoma occur
head or neck or genitourinary tract
subtype that arises in the mucosal lining of hollow organs and forms polypoid, grape like growth, has the best chance of survival
botryoid subtype
occurs in early to middle adolescence
alveolar rhadomyosarcoma
where does alveolar rhadomyosarcoma occur
deep soft tissue of extremities
what is alveolar rhadomyosarcoma based on
certain fusion genes
rarest type of rhadomyosarcoma that is in the deep soft tissue of adults
pleomorphic rhadomyosarcoma
2 tumors of smooth muscle
leiomyoma
where do most leiomyomas arise
the uterus
what are leiomyomas called
uterine fibroids (present in women)
what can leiomyomas arise from
erector pili muscles of skin, nipples, scrotum, labia
are leiomyomas malignant
no
malignant type of smooth muscle tumor
leiomysarcoma
where do leiomyosarcomas occur most frequently
uterus or GI tract
benign tumor of blood vessel that occurs at ANY SITE ON SKIN
hemangioma
benign tumor of blood vessel that is eruptive
pyogenic
benign tumor of blood vessel in skin and subcutaneous of soft tissue. may occur in neck or axilla of children
lymphangioma
what could cystic hygroma of neck in children be associated with
turner syndrome
benign tumor of blood vessel that is painful and shows modification of glomus body. Occurs in DISTAL DIGITS
glomus tumor and glomangioma
not true neoplasms, dilations of prexisitng vessels
vascular ectasia
usually on head or neck of infants
nevus flammeus (birthmark)
spider like seen w/excess estrogen
spider angiomas
autosomal dominant disorder w/vascular ectasias over skin, oral mucous membranes, respiratory, GI
hereditary hemorrhagic telangiectasia
benign tumor of blood vessel that is reactive vascular proliferation resulting from OPPORTUNISTIC INFECTION
bacillary angiomatosis
what causes bacillary angiomatosis
infection w/bartonella bacteria in immunocompromsied
where do bacillary angiomatosis lesions occur
anywhere
treatment for bacillary angiomatosis
antibiotics
malignant tumor of blood vessels that is aggressive
angiosarcoma
who is affected by angiosarcomas
older adults
presentation of angiosarcomas
skin of head and neck, scalp, soft tissue, breast, liver
what could angiosarcoma be secondary to
radiation therapy
what is seen with angiosarcomas
dilated lymphatics
less aggressive malignant blood vessel tumor and is very rare
hemangioendothelioma
infectious sarcoma
kaposis sarcoma
cause of kaposis sarcoma
HHV 8
virus in concert w/cofactor leads to…
endothelial cell proliferation
4 types of kaposi sarcoma
AIDS asscoiated
2 peripheral nerve tumor-like lesions
Morton neuroma
caused by repeated minor trauma, typically occurs in planter nerve between third and fourth toes
morton neuroma
occurs when peripheral nerve is severed or crushed
traumatic neuroma
3 types of peripheral nerve sheath tumors
schwannoma
arises from schwann cell and is benign
schwannoma
what may schwannoma be associated with?
neurofibromatosis type 2 (NF2)
benign tumor in discrete localized mass
neurofibroma
what does presence of multiple neurofibromas or plexiform neurofibromas result in or suggest?
diagnosis of neurofibromatosis type I
type of disorder of neurofibromtosis
autosomal domiant
what do the mutations act as
growth suppressors
what type is more common
type I
type I mutation
codes for neurofibromin on chromosome 17
peripheral type
type I
what is seen with type I neurofibromatosis
cafe au late spots
type II mutation
codes for merlin
what does merlin do
regulates membrane receptor signaling on chromosome 22
central type
type 2
what is type 2 associated with
bilateral acoustic schwannomas
where do malignant peripheral nerve sheath tumors occur
nerve trunks or proximal portions of upper and lower extremities or trunk
what do 50% of MPNSTs occur with
NF1
highly malignant sarcoma
MPNST
lesions of synovial origin
tenosynovial giant cell tumor
benign tumor either localized or diffuse characterized by fusion gene abnormality
tenosynovial giant cell tumor
presents as solitary and slow growing, involves tendon sheaths of fingers and wrists
localized form of tenosynovial giant cell tumor
most common mesenchymal neoplasm of hand
localized tenosynovial giant cell tumor
presents in knee followed by ankle and foot, rare
diffuse form of tenosynovial giant cell tumor
doesn’t show synovial cell differentiation and is uncommon in joint cavity
synovial sarcoma
where does synovial sarcoma present
para articular regions of extremites, less common in head and neck
age group affected w/synovial sarcoma
adolescents and young adults
epithelial and spindle cell components
biphasic type of synovial sarcoma
spindle cell component only of synovial sarcoma
monophasic type
what test is helpful in diagnosis of synovial sarcoma
immunohistochemistry
what is immunohistochemistry postive for with synovial sarcoma
cytokeratin (epithelial marker)
abnormal fusion in synovial sarcoma
SYT-SSX
extracellular MUCIN accumulation assoicated with cellular proliferation
myxomatous lesions
examples of myxomatous lesions
intramusclar myxoma
common myxomatous lesion that isn’t a neoplasm, occurs in fibrous connective tissue almost always near joint capsule or tendon sheath
ganglion cyst
most common location of myxomatous lesion
wrist
what are lesions known as on the fingers
digital mucous cysts
most common mesenchymal lesion of the hand and wrist
ganglion and digital mucus cyst
common benign lesions
lipomas
common adult sarcomas
undifferentiated pleomorphic sarcoma
common childhood sarcoma
rhabdomyosarcoma
