Tumor OITE 1 Flashcards
tx of chondroblastoma
intralesion currettage and bone grafting - can recurr and can mets to lung
chemo works on which malignant soft tissue sarcoma
rhabdo and synovial
labs in Pagets
high ALP; Hydroxyproline (Collagen marker); and increased Urinary N and C-telopeptide
what expresses S100
Nerve Sheet tumors (schwanomma, neurofibroma, MPVNST), clear cell sarcoma, liposarcoma,
vinca alkaloid (vincristine/blastine)
give HOT compress and hyaluronidase (cold compress for other vesicants)
which soft tissue sarcoma go to regional lymph nodes
synvoical, rhabdo, clear cell, epitheloid
tx of enchondroma
curettage and bone grafting
presentation of pagets sarcoma
pain, soft tissue swelling; or frx; avg age is 55-80
chronic sinus lead to what klind of cancer
SQUAMOUS cell - marjolin ulcer
tx of rhabdomyosarc
chemo only if widespread mets; rads for unresectable or close margins; usually tx with wide exision and chemo for peds - use vincristine and dactinomycin and c-phosphamide; wide excision + rads is for adults
when to worry about enchondroma
if getting bigger on films; lytic change; advanced scalloping
pathology in osteopetrosis
dysfunctonal osteoclasts - no remodeling
chondrosarcoma histology
lots of chondrocytes in no good pattern; blue balls of cartilage
most common site for mets
Lung > Liver> bone (t-spine)
desmoid tumor on MRI
dark to minimally bright
GCT in pevlis tx
needs XRT, 15% rate of malignancy
tx of abc fracture
non-op
most common benign bone tumor in kids
NOF - look for bubbly lesion with sclerotic rim
Blastic percentage rates
Lung Breast Prostate - 30, 60, 90% blastic rates
synovial sarc gene
x:18; SYT-SSX 1 or 2 fusion protein
keratin stain seen in
epiotheloid sarcoma, synovial sarcoma, any carcinoma, 50% of epitheloid angiosarcoma, rate in other mesenchymal tumors (leiomyosarc)
Paget’s Sarcoma
very rare secondary tumor 1%; usually 50-80s; 5y survival is < 10%; tx is surg + chemo
pagets and THA
increased EBL - can give pre bisphos to help
what is the only sarcoma that responds well to chemo
RHABDOsarcoma - still needs surg
CD20
bcell (lymphoma)
gene in fibrous dysplasia
FGF23 - GS alpha protein on C20; in cAMP pathway
fxn of doxorubicin in chemo
blocks DNA-RNA synthesis via blocking topoisomerase II - leads to apoptosis
osteopetrosis
marble bone diseaes - increased density and bone size - look for metaphyseal flair (flask deformity);
atypical lipomatous tumor
well differentiated low grade liposarc - marginal excision as well
how to distinguish osteopoikilosis vs malignant carcinoma
bone scan - osteoP is COLD
tx of giant cell
lesional curettage and LOCAL adjuvant (any will do - no diff in bisphos, liquid Nitrogen etc)
what mAb is approved for GCT
denosumab - antibody to RANKL
vimentin
all mesenchymal soft tissue tumors; also seen in lymphoma, melanoma, and some carcinoma (renal, enodmetrial and thyroid), - not super useful
ABC histo
lake of blood cells in a cystic space
chondrosarcoma tx
WIDE excision ONLY
chondromyxoid fibroma
rare, seen in kids, often in the knee at prox tib/fib or distal femur - can occur in wrist; tx w/ excision or curretage + PMMA; benign tumor that is locally aggressive -myxoid and fibrous elements
liposarc poor prognositc factors
age > 45; > 25% round cell, and tumor necrosis
poor prognostic factors in Ewings
p53 mutation and high LDH
liposarcoma HPI
40-60y; deep lesions in retroperitoneum
what does osteoid osteoma present lots of
PGEE2 and I2
all Neurofibroma lesions stain for
S100
osteosarcoma on bone scan
HOT
Fibrous Dysplasia
benign, poly or mono-ostotic; ground glass on XR;
XRT a/w
high grade Sarcoma
Chordoma
85% in sacrum or C-spine - MIDLINE lesion;
Harringtons criteria
> 50% diaphyseal destruction; >50-75% destruction of metaphysis, destruction of subtroch region, pain despite rads
bubbly lesion in bone
desmoid tumor - aka desmoplastic fibroma
tx of ewings
chemo, surg, chemo (wide excision) - about 70% survival with chemo surg
rads tx of HO prophylaxis
700cGy within 72 hrs of surg
myxoid liposarc is dark on T1 bc
less than 10 % mature fat
Anterolateral bowing a/w
Neurofibromatosis
use of bisphos in
Mets; Myeloma, Pages, Polyostotic fibrous dysplasia
tx of Bcell lymphoma
usually chemo; surg only for skeletal integrity (25% have path frx); survival is about 70%
PVNS HPI
knee area; slowly growing
Histo of Eosino G
look for langerans histiocytes; kidney bean nuclei; Bierbeck body; CD1a stain;
lytic appear osteosarc
telangiectactic
tx of adult chondrosarc
Wide Resection alone if intramedullary; often found in scapula, pelvis or prox femur
PNST on MRI
BRIGHT on T2; mixed target appearance on T1
histo of leiomyosarcoma
stains + for smooth muscle actin; high mitotic activity; lots of cellular variability
Most common lytic destructive mets come from
Lung then Renal
prognosis of chordoma
long term is 25-50%; local recurrence is a problem - need to preserve sacral roots for good QoL
tx of lymphoma
stabilize and chemo +/- radiation
POEMS lesion
polyneuropathy, oraganomegaly, endorcinopathy, and M protein spike with skin changes - tx w/ XRT
age group of Giant Cell
20-40’s - usually extends to subchondral; but in kids usually stays on metaphysis
Paget’s
abnoral bone REMODELING- problem with osteoclasts; seen in 50’s, 3 phases (lytic, mixed resorp and compenstaion phase, and scerlotic phases from osteoblastic bone) - all 3 phases can occur in same bone
intramuscular myxoma looks like on histo
nuclei + fat cells; lots of open spaces in cells - homogenous dark on T1 MRI, bright on t2
tx of GCT
currettage with adjuvant (phenol, PMMA freeze etc) +/- bone grafting
when GCT in spine where
VT body -not post elements
tx of ABC
curettage and phenol or cryo - can excise if bone is expendable; XRT for unresectable
most common soft tissue sarcoma in child
rhabdomyosarcoma
ABC on MRI
fluid levels; but cannot ignore telagniectatic osteosarcoma
cyst recurrence from UBC
common enough; more likely if within 2 cm of physis
stains for rhabdomyosarc
vimentin, desmin, myosin
PNST Neurofibroma histo
loose wave cells with strand like collagen; leson is the nerve - cannot excise
synovial sarc presentation
thigh or shoulder girldle; look for calcificiations; 25% spread via lymph; most common malignant soft tissue sarcoma OF LOWER EXTREMITY
PVNS histology
synovial cells; hemosiderin, giant cells, cleft like patterns,
CD 38
Plasma cell antigen (myeloma or plasmacytoma)
ABC vs telangiectacti osteosarc
tx is curettage for ABC and chemo/surg for T. osteosarc - need to distinguish via histo - bone on the osteosarc
labs in Multiple myelooma
hyperCa; M spike (IgG or IgA); high Creatinine
pagets fractures
the heal but SLOWLY; and never regain normal strength
risk of malignancy for chondromas
Mafucci (50%); Ollier(10%); MHE (10%); Single enchondroma (1%); single osteochondroma (< 1%)
what MUST you do for rhabdomyosarc work up
bone marrow aspirate
what is a low grade osteosarc
PAROSTEAL - tx with wide excision ONLY
Ewings sarcoma XR findings
moth eaten, NO soft tissue mineralization, big soft tissue mass, XR shows sucked on bone
gene in diastrophic dysplasia
DTDST (sulfate transporter); look for caulifolower ears
synovial chondromatosis
chondroi loose bodies in joint from synovial metaplasia; the process burns out over time
synovial sarcoma grows
SLOW
Ewings on MRI
Dark on T1, Bright on T2
osteoblastoma features
similar to osteoid osteoma but > 2cm; loves the POST elements of the spine; lytic in spine; blastic in extremities; HOT on bone scan; no bone reaction but lots of soft tissue reaction seen on MRI
when do you radiate GCT
if unresectable for curretage
osteopoikilosis
autodom condition of multiple bone islands in cancellous bone - COLD on bone scan
Adamantinoma tx
wide intercalary resection; look for epitheloid cell nests on histo