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
tx of osteoblastoma
extended intralsional currettage
Peripheral nerve sheath tumor (Neurilemoma)
aka benign schwanoma - seen on flexor surfaces, slow growing and asymptomatic; spares the bundles of a nerve so can be excised
what med prevents formation of lytic lesions
bisphosphonates
NOF
benign eccentric lesion; in metaphysis of long bones; look for bone island after healing;
NOF
eccentric, multi-loculated subcortical lesions with central lucency and scalloped sclerotic margin
Fibrosarcoma HPI
any location but usually LE; older patients;
chondroBLASTOMA
lytic in epiphysis ages 10-14; or acetab or hind foot in adults; 25-30% have flecks of calcifications;
PNST - neurofibroma
CANNOT be excised bc its part of nerve bundles; 20-30year old patients;
NF1 vs NF 2
NF 1 is peripheral; Lisch nodules; and tibial pseudoarthrosis C17 -neurofibrillin defect; NF 2 has CN 8 neurofibromas C22
chondrosarc HPI
painful and Slow growing - distal femur prox humerus or periacetbular at times
gene for alveolar rhabdomyosarco
Pax3-FHR - fusion protein; a/w high risk of mets - t(2,13)
tumor induced bone lysis
PTHrP in breast stimulates OsteoB to release RANKL –> tells OsteoC to resorb
Rhabdomyo sarc on MRI
Dark on T1, Bright on T2 - often head/neck or GU, retroperitoneum
Garder syndrome a/w
low grade fibrosarcoma
osteofibrous dysplasia confused with
adamantinoma (might be a precursor) - but this one is benign and also on ant tib cortex; + rimming osteoblasts
fibrous dysplasia histology
fibrous osteoid; no rimming osteoblasts; chinese letters (c’s and o’s) with spindle cell fibrous tissue
worst prognosis after bone mets
Lung, then Melanoma both are < 6 months; 5y survival is 5%
B Cell lymphoma
35-55 y adults; NHL any age; permeative lesion in long bone of adults; VERY HOT ON BONE SCAN - CD20 AND CD45
chondroblastoma histo
chicken wire; cobblestone; S100 stain
CD34
vascular tumors (benign and malignant) - hemangioma angiosarcoma, eiptheloid sarcoma
tx of fibrous dysplasia
nothing if no fracture
what predicts local recurrence and mets in chondrosarcoma
histologic grade; look for large chondrocytes with nuclear atypia
most common tumor of infancy and childhood
hemangioma - usually can tx with perc sclerosing agent
most common soft tissue sarcoma of lower extremities
SYNOVIAL sarcoma
tx of clear celll
amputation and chemo - proximal lesions can be tx with wide resection and radiation
hemangioma on MRI
wormy appearance on T2; T1 mixed dark with bright fat; can be in muscle; often seen in vertebral body (jail house)
rhabdomyosarc surviviall rates
Embryonal 80%; Alveolar 60%; Botryoid 30%; Pleo 25%
antidote for mechlorethamine and cisplatine
sodium thiosulfate
tx of rhabdomyosarc
chemo, surg and XRT
failure rate of RF ablation on osteoid osteoma
15%
tx of osteoblastoma
currettage and grafting
osteophyllic carcinomas
Breast, Lung, Thyroid, Renal, Prostate
SUPER HOT on bone scan
pagets
what stains for vimentin and Epithelial membrane antigen
Synovial cell sarcoma - often shows calcificiations - tx with wide resection and chemor or rads
tx of NOF
obs ; curettage and bone graft if symptomatic;
what percent of mets have drug pumps
50%; (25% in primary cancers)
when to worry about osteochondroma
if > 2cm then needs more surveilance; or if growing after skeletal maturity
lipoblastoma
infants get this; respond with marginal excision;
NOF histo
swirling fibrous tissue with giant cells;
café au lait seen in NF
coast of california
tx of pagets
supportive tx; if symptomatic then bisphos (or calcitonin if they cant tolerate)
Chondrosarcoma survival rates
Grade I 90%; II 60-70%; III 30-50%; dedifferentiated is 10%
Ewings stain
no effect on survivall, only local recurrence
gene in pagets
5q35 QTER gene
mets of synovial sarc go to
lung 30-60%
osteoid osteoma on bone scan
HOT
PNST (neurilemoma) histo
verocay bodies (nuclear pallisading); Antoni A(cellular); Antoni B(myxoid)
synovial sarcoma survival linked to
SIZE
Cold on Bone Scan
Renal, Thyroid, Multiple Myeloma (and eosinophillic granuloma)
work up Multiple myeloma
labs+ BM aspirate for marow involvment; >10% plasma cells (minor criteria); or > 30% major criteria, - normal is 2-3% plasma cells
Mafucci syndrome
multiple enchondroma and hemangioma
management of asymptomatic enchondroma
obs
UBC in calc
will shrink to skeletal maturity and eventually go away
general tx of high grade soft tissue sarcoma
Rads + Surg. Chemo only if systemic or unresectable
tx of fracture from UBC
sling if proximal humerus (50-75% p/w fracture); can tx other symptomatic lesions with intralesional steroid or bone marrow, curettage and bone graft with internal fixation if needed
Clear Cell Sarcoma HPI
20-40yo; in feet; dark lesion a/w tendons; dorsum of foot; poor prognosis
clear cell histo
clear cytoplasm, stains for S100; HMB45; c12:22 translocation; spindle cells throughout
Multiple myeloma HPI
pain is a LATE finding; needs a skeletal survey;
tx of osteosarc is
chemo, wide excision, chemo and recon
fibrous dysplasia in front of tibia is
osteofibrous dysplasia - observation only; on histo look for osteoblast rimming
how to distinguish a malignant chondroid lesion
larger (>8cm); older age, central skeleton, night pain, local recurrence, hot on bone scan, axial skeleton (vs appendicular)
Leiomyosarc histo
cigar nuclei; lots of pink cytoplasm; stain for desmin/actin and vimentin (sarcoma marker)
what tumors express desmin
rhabdomyosarcoma
recurrance for chondrosarc related to
increased telomerase activtity
poor prognostic factors in Ewings
spine, pelvic tumors, large size, < 90% necrosis on chemo, high LDH, p53 mutation
CD 99
Ewings /PNET - can use in synovial sarcoma too
CD1a
Eosinophilic granuloma; histiocytic lesions
fibrous dysplasia heals with
fibrous dysplasia
tx of liposarcoma
rads and surg
multiple NOF part of what syndrome
Jafffe capanacci - includes vision problems, Mental retardation; and café-au-lait
PVNS
hemosiderin, INTRA articular; - the non-synovial version of this is giant cell tumor of tendon sheath
myxoid liposarc gene
t12:16
Leiomyosarc found in
retroperitoneum usually but can be in extremities - arise from Smooth muscle
tx of leiomyosarc
wide resection and XRT - poor prognosis
osteoid osteoma histo
big ring of bone with tiny cells and mucoid in the middle
tumoral calcinosis
rare; in African american women; a/w metabolic defecfts, collagen disorders, and trauma - look for calcific debris on xray that’s lobular
tx of chordoma
wide resection; if unresectable or margins are questionable then XRT
radiation effect on soft tissue sarcoma
no effect on survivall, only local recurrence
multiple myeloma stain
CD38
Epitheloid sarcoma HPI
young adults; hand or forearm - nodular on histology; lots of eosinophillic cytoplasm; can spread via lymph
multiple fibrous dysplasia and endocrin issues
Albright’s syndrome
low grade chondrosarc adjuvant
phenol or argon beam with grafting if its a low grade chondrosarcoma
BCR ABL is
philadelphia c=some in CML
rhabdomyosarc HPI
most common under age 15; embryonal is most common overall and in kids; alveolar is most common in extremities;
osteosarc limb salvage vs amp
equivalent outcomes so choose limb salvage
Ewings gene
EWS-FL1
tx for myxoid liposarc
wide excision and XRT
Bright T1
Fat, Marrow, Proteinaceious Fluid, Gadolinium, Met Hg, Melanin,
tx of desmoid tumor
usually chemo and nsaids for large lesions; if surg then WIDE margins bc of reccurrence issues +/- XRT
what is side effect of doxorubicin
cardiac toxicity; dexrazoxane used to mediate the toxicity
why cant you use bone scan for stress fracture
wont be hot until at least 72 hrs after
melorheostosis
waxed bony run off - HOT on bone scan
Ewings workup requires
bone scan, bM biopsy, and Chest CT
stains for rhabdomyosarc
MyoD1; Myoglobin, myosin, desmin, and vimentin
tx of pagets sarcoma
chemo; wide resect; recon
4 types of rhabdomyosarcoma
embryonal (in infants, young children); alveolar (teens and young adults); botryoid (infants and young children - near vagina - bunch of grapes on path); pleomorphic in 40-70’s
in patient with pagets what are you at risk of
osteosarcoma
histo of pagets looks like
osteoblastoma
soft tissue calcifications
synovial sarcoma - look for CENTRAL Ca for malignant stuff
prognosis of synovial sarcoma linked to
size <5cm all survived; 5-10cm 75%; and >10 cm 20% survival
myoD1 and myogenin Myf 4 stain found in
skeletal muscle
most common soft tissue sarcoma in adults overall
High Grade Spindle Cell Sarcoma
Neuroblastoma
INFANTS - look for large adrenal mass on CT scan or bone scan; stain + for NB84; true rossets (circular cell pattern)
what med can you give with GCT
denosumab - antibody to RANKL
Eosinophillic Granuloma
COLD on bone scan; 10-20’s of life; PAIN is COMMON; lesions show up in skull, ribs and long bones - veretebral plana lesions will heal
histo of talangiectatic osteoS
dilated blood filled vascular space lined by malignant osteoblasts
3 stages of GCT
capanacci: 1 in bone; II expands bone with rim; III into soft tissue
chordoma looks like _ on histology
liposarcoma - massive bubble cells
synovial chromatosis can progress to
synovial chondrosarcoma
CD 31
most specific endothelial maerk - stains normal endothelail cells
enchondroma
Long, oval, with well defined margin - look for thinned cortex that has expanded; small round nuclei with chondrocytes
what cx is Rads and Chemo RESISTENT
classic intramedullary CHONDROSARCOMA; tx is wide surgical excision
Chondromyxoid sarcoma gene
EWS-CHN 9;22
desmoid tumor (fibromatosis)
extra abdominal lesion; shouilder is most common site;
stains for synovial sarc
vimentin, keratin, and Epith Membrane antigen
café au lait seen in in Albrights
coast of maine (ragged)
tx of epitheloid sarc
usually wide resection but may need amputation as this travels up the arm
tx of osteopetrosis
Bm transplant
EMA (epitheloid membrane antigen)
epitheloid sarcoma, synovial sarcoma,
signet ring cell seen with
liposarcoma
tx of PVNS
simple excision vs complete synovectomy; the extra articular analogy is GCT of tendon sheath
Malignant PNST
50% start from PNST; tx w/ resection and XRT - prognosis is < 20%
histo of High grade spindle cell sarcoma
pleomorphic and storiform - herringbone pattern ‘ tx is surg + XRT; chemo if systemic; poor prognosis
xray findings of chondrosarcoma
look for scalloping of the cortex with periosteal reaction
Gauchers disease bone deformity
ALSO erlenmeyer flask deformity bc of gluccocerebrosides in marrow; tx with enzyme replacement (glucocerebrosidase)
tx of fibrous dysplasia
obs for asx lesions; otherwise curettage and bone graft
most common soft tissue sarcoma in foot
Synovial cell sarcoma - often shows calcificiations
NF -1 a/w with which tumor
MPNST
simply bone cyst
90% are prox femur or prox humerus; usuall tx with obs and then deal with cyst; look for fallen frag of cortex into cyst;
Tx of MM
chemo and XRT +/- bisphos to preent new lytic lesions
EXT 1 vs EXT 2
EXT 1 has higher rate of sarcoma transformation; more limb malalignment, shorter segments and more pelvic-flat bone involvment
overal MOST common soft tisuse sarcoma
LIPOSARCOMA
giant cell tumor on MRI
dark on T1
MHE with EXT 1 VS 2
EXT 1 has more severe presentation; error in chondrocyte proliferation; MHE have risk of sarcoma transformation - EXT genes code for exostosisn which are involved in biosynthesis of hep sulfate
Blue Cell Tumors
lymphoma, ewings, Rhabdo, neuroblastoma, myeloma
osteoblastoma histo
wavy thick bone with cells in b/w
osteosarc survival without chemo
17%
periosteal chondroma HPI and xr
painful in prox humerus - seen as small lytic lesion INSIDE the cortex - projects into soft tissue space
