B6.016 - Soft Tissue Tumors Prework Part 1 Flashcards
what are soft tissue tumors
mesenchymal proliferations that occur in nonepithelial, extraskeletal tissues of the body most are benign
describe where soft tissue tumors occur in the body
40% lower extremities, 20% upper extremities, 10% head and neck, 30% trunk and retroperitoneum
3 categories of soft tissue tumors
- benign 2. intermediate (borderline), frequently recur and rarely metastasize 3. malignant (sarcoma)
describe treatment of sarcomas
depends on:
Type/site
grade
stage
what are the classifications of soft tissue tumors (adipose, fibrous tissue, smooth muslce)
- tumors of adipose tissue
- Lipoma
- Atypical lipomatous tumor
- Liposarcoma
- Tumors and tumor like lesions of fibrous tissue
- fibroma, nodular fasciitis
- fibromatoses
- fibrosarcoma
- Tumors of smooth muscle
- leiomyom
- leiomyosarcoma
what are the tumors of skeletal muscle
Rhabdomyoma
rhabdomyosarcoma
what are the vascular tumors
hemangioma
kaposi sarcoma
angiosarcoma
what are peripheral nerve sheath tumors
neurofibroma, schwannoma
malignant peripheral nerve sheath tumor
what are the tumors of uncertain histogenesis
synovial sarcoma
undifferentiated pleomorphic sarcoma
describe typical ages for rhabdomyosarcoma, synovial sarcoma, liposarcoma
rhabdomyosarcoma - children
synovial sarcoma - young adults
liposarcoma - mid to late adult life
important prognostic information for tumor
size, mitotic activity, degree of necrosis
lipoma features, tx, histo
benign tumor of fat, most common soft tissue tumor of adults
soft, mobile, usually painless
histo - encapsulated mass of mature fat cells
cured by simple excision
lipoma
liposarcoma features, where it arises
one of the most common sarcomas in adults
arise in deep soft tissues of proximal extremities and retroperitoneium
subtypes of liposarcomas
- well differentiatied (indolent)/dedifferentiated (aggressive) (Amplif. of MDM2 oncogene)
- myxoid/round cell (t(12;16)(q13;11);fusion with DDIT3(CHOP) gene with FUS)
- pleomorphic liposarcoma (aggressive)
liposarcoma histology
lipoblasts are almost always present
resemble fetal fat cells
contain cytoplasmic vacuoles of lipid that scallop the nucleus
wide excision necessary
Well differentiated liposarcoma
has lipoblasts and MDM2 amplification
well differentiated liposarcoma
has lipoblasts and MDM2 amplification
dedifferentiated liposarcoma
has MDM2 amplification, arises from WD liposarcoma, higher grade
Myxoid liposarcoma
t(12;16)(q13;11), DDIT3-FUS fusion
arrows pointing to lipoblasts
myxoid liposarcoma
t(12;16)(q13;11), DDIT3-FUS fusion
nodular fasciitis features
reactive pseudosarcomatous proliferation (sarcoma LIKE)
presents with recent hx of solitary rapid growing sometimes painful mass
often occurs in adults on the volar aspect of the forearm, chest and back
arises in deep dermins, subcutis or muscle, nodular and poorly defined
histo of nodular fasciitis
plump, randomly arranged spindle cells (fibroblasts) in a myxoid background containing lymphocytes and extravasated RBCs. Abundant mitotic figures.
May regress spontaneously
rarely recurs after excision
nodular fasciitis
types of superficial fibromatosis and histo appearance
palmar (dupuytrens contracture)
plantar (ledderhosens disease)
penile (peyronies disease)
histo: nodular or poorly defined bundles of mature fibroblasts surrounded by abundnat dense collagen
superficial fibromatosis
describe deep seated fibromatosis
desmoid tumors
more aggressive than superfiical ones
more common in females
divided into extra abdominal, abdominal and intra
mutations in beta catenin genes are present in majority
intra abdominal desmoids often occur in patients having
gardner syndrome, variant of familial adenomatous polyposis
histo/gross appearance of deep seated fibromatosis (desmoid tumors), tx
grossly: grey/white, firm, infiltrative masses
histo: long fascicles of elongated fibroblasts and collagen fibers, IHC: B-catenin
locally aggressive, recur if incompletely excised. Dont metastasize
may respond to tamoxifen, chemo or irradiation
desmoid tumor
desmoid tumor
desmoid tumor
fibrosarcoma features, histo
malignant fibrous tumor
rare sarcoma, occurs mostly in retroperitoneum, thigh, knee and distal extremities
histo: typical composed of malignant spindle cells arranged in herringbone pattern with frequent mitoses and areas of necrosis
aggressive with high recurrence rate, may metastasize
fibrosarcoma
leiomyoma fetures and histo
benign tumor of smooth muscle
very common in uterus
histo: intersecting bundles (fascicles) of spindle cells with blunt ended cigar shaped nuclei, minimal atypica and few mitoses
treated by simple surgical removl
leiomyoma
leiomyoma
leiomyoma
features, histo of leiomyosarcoma
more often in women than men
most develop in skin, deep soft tissues of limbs and retroperitoneum
histo: malignant spindle cells with cigar shaped nuclei, frequent mitoses and necrosis
IHC: smooth muscle actin (SMA), H-caldesmon
leiomyosarcoma
leiomyosarcoma
leiomyosarcoma
leiomyosarcoma
rhabdomyosarcoma features, histo, IHC
most common soft tissue sarcoma of children and adolescents (<20)
most occur in head and neck or GU
histo: malignant cell is rhabdomyoblast (aka strap cell)
exccentric nucleus iwht abundnat granular eosinophilic cytoplasm with cross striations
IHC: Desmin, myoglobin, MyoD1
rhabdomyosarcoma
rhabdomyosarcoma
rhabdomyosarcoma
3 types fo rhabdomyosarcoma
- embryonal
- alveolar
- pleomorphic
compare embryonla, alveolar, pleomorphic rhapbdomyosarcoma based on site, epidemiology, and clinical appearance
embryonal rhabdomyosarcoma features
most common (70%)
develops in wall of hollow mucosal lined organs, like nasopharynx, bile duct, vagina and bladder
children <5 yo
sarcoma botryoides is most common variant “bunch of grapes”
no characteristic cytogenic abnormality
sarcoma botryoides
rhabdomyosarcoma
botryoid RMS
botryoid RMS
sarcoma botryoides
RMS
alveolar RMS features
aggressive, 30%
deep muscles of extremities, head and neck, 10-25 yo
t(2;13) (q35;q14)PAX-FOXO1 translocation
FISH detects FOXO1 breakpoint
PCR detects FOXO1-PAX fusion transcripts
what does PAX3-FOXO1 protein do
feature of alveolar RMS
chimeric protein leads to dysregulation of skeletal muscle differentiation
histo of alveolar RMS
high grade small round cell tumor, solid alveolar growth pattern, central loss of cohesion
rhabdomyoblasts may be seen
alveolar RMS
alvolar RMS
alvolar RMS
alveolar RMS
alveolar RMS
tx of RMS and ranking of prognosis best>worst
surgery, radiation, chemo
botryoid>embryonal>pleomorphic>alveolar
