Tumor OITE 1 Flashcards

1
Q

tx of chondroblastoma

A

intralesion currettage and bone grafting - can recurr and can mets to lung

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2
Q

chemo works on which malignant soft tissue sarcoma

A

rhabdo and synovial

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3
Q

labs in Pagets

A

high ALP; Hydroxyproline (Collagen marker); and increased Urinary N and C-telopeptide

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4
Q

what expresses S100

A

Nerve Sheet tumors (schwanomma, neurofibroma, MPVNST), clear cell sarcoma, liposarcoma,

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5
Q

vinca alkaloid (vincristine/blastine)

A

give HOT compress and hyaluronidase (cold compress for other vesicants)

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6
Q

which soft tissue sarcoma go to regional lymph nodes

A

synvoical, rhabdo, clear cell, epitheloid

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7
Q

tx of enchondroma

A

curettage and bone grafting

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8
Q

presentation of pagets sarcoma

A

pain, soft tissue swelling; or frx; avg age is 55-80

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9
Q

chronic sinus lead to what klind of cancer

A

SQUAMOUS cell - marjolin ulcer

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10
Q

tx of rhabdomyosarc

A

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

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11
Q

when to worry about enchondroma

A

if getting bigger on films; lytic change; advanced scalloping

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12
Q

pathology in osteopetrosis

A

dysfunctonal osteoclasts - no remodeling

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13
Q

chondrosarcoma histology

A

lots of chondrocytes in no good pattern; blue balls of cartilage

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14
Q

most common site for mets

A

Lung > Liver> bone (t-spine)

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15
Q

desmoid tumor on MRI

A

dark to minimally bright

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16
Q

GCT in pevlis tx

A

needs XRT, 15% rate of malignancy

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17
Q

tx of abc fracture

A

non-op

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18
Q

most common benign bone tumor in kids

A

NOF - look for bubbly lesion with sclerotic rim

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19
Q

Blastic percentage rates

A

Lung Breast Prostate - 30, 60, 90% blastic rates

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20
Q

synovial sarc gene

A

x:18; SYT-SSX 1 or 2 fusion protein

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21
Q

keratin stain seen in

A

epiotheloid sarcoma, synovial sarcoma, any carcinoma, 50% of epitheloid angiosarcoma, rate in other mesenchymal tumors (leiomyosarc)

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22
Q

Paget’s Sarcoma

A

very rare secondary tumor 1%; usually 50-80s; 5y survival is < 10%; tx is surg + chemo

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23
Q

pagets and THA

A

increased EBL - can give pre bisphos to help

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24
Q

what is the only sarcoma that responds well to chemo

A

RHABDOsarcoma - still needs surg

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25
Q

CD20

A

bcell (lymphoma)

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26
Q

gene in fibrous dysplasia

A

FGF23 - GS alpha protein on C20; in cAMP pathway

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27
Q

fxn of doxorubicin in chemo

A

blocks DNA-RNA synthesis via blocking topoisomerase II - leads to apoptosis

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28
Q

osteopetrosis

A

marble bone diseaes - increased density and bone size - look for metaphyseal flair (flask deformity);

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29
Q

atypical lipomatous tumor

A

well differentiated low grade liposarc - marginal excision as well

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30
Q

how to distinguish osteopoikilosis vs malignant carcinoma

A

bone scan - osteoP is COLD

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31
Q

tx of giant cell

A

lesional curettage and LOCAL adjuvant (any will do - no diff in bisphos, liquid Nitrogen etc)

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32
Q

what mAb is approved for GCT

A

denosumab - antibody to RANKL

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33
Q

vimentin

A

all mesenchymal soft tissue tumors; also seen in lymphoma, melanoma, and some carcinoma (renal, enodmetrial and thyroid), - not super useful

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34
Q

ABC histo

A

lake of blood cells in a cystic space

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35
Q

chondrosarcoma tx

A

WIDE excision ONLY

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36
Q

chondromyxoid fibroma

A

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

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37
Q

liposarc poor prognositc factors

A

age > 45; > 25% round cell, and tumor necrosis

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38
Q

poor prognostic factors in Ewings

A

p53 mutation and high LDH

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39
Q

liposarcoma HPI

A

40-60y; deep lesions in retroperitoneum

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40
Q

what does osteoid osteoma present lots of

A

PGEE2 and I2

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41
Q

all Neurofibroma lesions stain for

A

S100

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42
Q

osteosarcoma on bone scan

A

HOT

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43
Q

Fibrous Dysplasia

A

benign, poly or mono-ostotic; ground glass on XR;

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44
Q

XRT a/w

A

high grade Sarcoma

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45
Q

Chordoma

A

85% in sacrum or C-spine - MIDLINE lesion;

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46
Q

Harringtons criteria

A

> 50% diaphyseal destruction; >50-75% destruction of metaphysis, destruction of subtroch region, pain despite rads

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47
Q

bubbly lesion in bone

A

desmoid tumor - aka desmoplastic fibroma

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48
Q

tx of ewings

A

chemo, surg, chemo (wide excision) - about 70% survival with chemo surg

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49
Q

rads tx of HO prophylaxis

A

700cGy within 72 hrs of surg

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50
Q

myxoid liposarc is dark on T1 bc

A

less than 10 % mature fat

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51
Q

Anterolateral bowing a/w

A

Neurofibromatosis

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52
Q

use of bisphos in

A

Mets; Myeloma, Pages, Polyostotic fibrous dysplasia

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53
Q

tx of Bcell lymphoma

A

usually chemo; surg only for skeletal integrity (25% have path frx); survival is about 70%

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54
Q

PVNS HPI

A

knee area; slowly growing

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55
Q

Histo of Eosino G

A

look for langerans histiocytes; kidney bean nuclei; Bierbeck body; CD1a stain;

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56
Q

lytic appear osteosarc

A

telangiectactic

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57
Q

tx of adult chondrosarc

A

Wide Resection alone if intramedullary; often found in scapula, pelvis or prox femur

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58
Q

PNST on MRI

A

BRIGHT on T2; mixed target appearance on T1

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59
Q

histo of leiomyosarcoma

A

stains + for smooth muscle actin; high mitotic activity; lots of cellular variability

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60
Q

Most common lytic destructive mets come from

A

Lung then Renal

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61
Q

prognosis of chordoma

A

long term is 25-50%; local recurrence is a problem - need to preserve sacral roots for good QoL

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62
Q

tx of lymphoma

A

stabilize and chemo +/- radiation

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63
Q

POEMS lesion

A

polyneuropathy, oraganomegaly, endorcinopathy, and M protein spike with skin changes - tx w/ XRT

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64
Q

age group of Giant Cell

A

20-40’s - usually extends to subchondral; but in kids usually stays on metaphysis

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65
Q

Paget’s

A

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

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66
Q

intramuscular myxoma looks like on histo

A

nuclei + fat cells; lots of open spaces in cells - homogenous dark on T1 MRI, bright on t2

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67
Q

tx of GCT

A

currettage with adjuvant (phenol, PMMA freeze etc) +/- bone grafting

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68
Q

when GCT in spine where

A

VT body -not post elements

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69
Q

tx of ABC

A

curettage and phenol or cryo - can excise if bone is expendable; XRT for unresectable

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70
Q

most common soft tissue sarcoma in child

A

rhabdomyosarcoma

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71
Q

ABC on MRI

A

fluid levels; but cannot ignore telagniectatic osteosarcoma

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72
Q

cyst recurrence from UBC

A

common enough; more likely if within 2 cm of physis

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73
Q

stains for rhabdomyosarc

A

vimentin, desmin, myosin

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74
Q

PNST Neurofibroma histo

A

loose wave cells with strand like collagen; leson is the nerve - cannot excise

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75
Q

synovial sarc presentation

A

thigh or shoulder girldle; look for calcificiations; 25% spread via lymph; most common malignant soft tissue sarcoma OF LOWER EXTREMITY

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76
Q

PVNS histology

A

synovial cells; hemosiderin, giant cells, cleft like patterns,

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77
Q

CD 38

A

Plasma cell antigen (myeloma or plasmacytoma)

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78
Q

ABC vs telangiectacti osteosarc

A

tx is curettage for ABC and chemo/surg for T. osteosarc - need to distinguish via histo - bone on the osteosarc

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79
Q

labs in Multiple myelooma

A

hyperCa; M spike (IgG or IgA); high Creatinine

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80
Q

pagets fractures

A

the heal but SLOWLY; and never regain normal strength

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81
Q

risk of malignancy for chondromas

A

Mafucci (50%); Ollier(10%); MHE (10%); Single enchondroma (1%); single osteochondroma (< 1%)

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82
Q

what MUST you do for rhabdomyosarc work up

A

bone marrow aspirate

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83
Q

what is a low grade osteosarc

A

PAROSTEAL - tx with wide excision ONLY

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84
Q

Ewings sarcoma XR findings

A

moth eaten, NO soft tissue mineralization, big soft tissue mass, XR shows sucked on bone

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85
Q

gene in diastrophic dysplasia

A

DTDST (sulfate transporter); look for caulifolower ears

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86
Q

synovial chondromatosis

A

chondroi loose bodies in joint from synovial metaplasia; the process burns out over time

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87
Q

synovial sarcoma grows

A

SLOW

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88
Q

Ewings on MRI

A

Dark on T1, Bright on T2

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89
Q

osteoblastoma features

A

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

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90
Q

when do you radiate GCT

A

if unresectable for curretage

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91
Q

osteopoikilosis

A

autodom condition of multiple bone islands in cancellous bone - COLD on bone scan

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92
Q

Adamantinoma tx

A

wide intercalary resection; look for epitheloid cell nests on histo

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93
Q

tx of osteoblastoma

A

extended intralsional currettage

94
Q

Peripheral nerve sheath tumor (Neurilemoma)

A

aka benign schwanoma - seen on flexor surfaces, slow growing and asymptomatic; spares the bundles of a nerve so can be excised

95
Q

what med prevents formation of lytic lesions

A

bisphosphonates

96
Q

NOF

A

benign eccentric lesion; in metaphysis of long bones; look for bone island after healing;

97
Q

NOF

A

eccentric, multi-loculated subcortical lesions with central lucency and scalloped sclerotic margin

98
Q

Fibrosarcoma HPI

A

any location but usually LE; older patients;

99
Q

chondroBLASTOMA

A

lytic in epiphysis ages 10-14; or acetab or hind foot in adults; 25-30% have flecks of calcifications;

100
Q

PNST - neurofibroma

A

CANNOT be excised bc its part of nerve bundles; 20-30year old patients;

101
Q

NF1 vs NF 2

A

NF 1 is peripheral; Lisch nodules; and tibial pseudoarthrosis C17 -neurofibrillin defect; NF 2 has CN 8 neurofibromas C22

102
Q

chondrosarc HPI

A

painful and Slow growing - distal femur prox humerus or periacetbular at times

103
Q

gene for alveolar rhabdomyosarco

A

Pax3-FHR - fusion protein; a/w high risk of mets - t(2,13)

104
Q

tumor induced bone lysis

A

PTHrP in breast stimulates OsteoB to release RANKL –> tells OsteoC to resorb

105
Q

Rhabdomyo sarc on MRI

A

Dark on T1, Bright on T2 - often head/neck or GU, retroperitoneum

106
Q

Garder syndrome a/w

A

low grade fibrosarcoma

107
Q

osteofibrous dysplasia confused with

A

adamantinoma (might be a precursor) - but this one is benign and also on ant tib cortex; + rimming osteoblasts

108
Q

fibrous dysplasia histology

A

fibrous osteoid; no rimming osteoblasts; chinese letters (c’s and o’s) with spindle cell fibrous tissue

109
Q

worst prognosis after bone mets

A

Lung, then Melanoma both are < 6 months; 5y survival is 5%

110
Q

B Cell lymphoma

A

35-55 y adults; NHL any age; permeative lesion in long bone of adults; VERY HOT ON BONE SCAN - CD20 AND CD45

111
Q

chondroblastoma histo

A

chicken wire; cobblestone; S100 stain

112
Q

CD34

A

vascular tumors (benign and malignant) - hemangioma angiosarcoma, eiptheloid sarcoma

113
Q

tx of fibrous dysplasia

A

nothing if no fracture

114
Q

what predicts local recurrence and mets in chondrosarcoma

A

histologic grade; look for large chondrocytes with nuclear atypia

115
Q

most common tumor of infancy and childhood

A

hemangioma - usually can tx with perc sclerosing agent

116
Q

most common soft tissue sarcoma of lower extremities

A

SYNOVIAL sarcoma

117
Q

tx of clear celll

A

amputation and chemo - proximal lesions can be tx with wide resection and radiation

118
Q

hemangioma on MRI

A

wormy appearance on T2; T1 mixed dark with bright fat; can be in muscle; often seen in vertebral body (jail house)

119
Q

rhabdomyosarc surviviall rates

A

Embryonal 80%; Alveolar 60%; Botryoid 30%; Pleo 25%

120
Q

antidote for mechlorethamine and cisplatine

A

sodium thiosulfate

121
Q

tx of rhabdomyosarc

A

chemo, surg and XRT

122
Q

failure rate of RF ablation on osteoid osteoma

A

15%

123
Q

tx of osteoblastoma

A

currettage and grafting

124
Q

osteophyllic carcinomas

A

Breast, Lung, Thyroid, Renal, Prostate

125
Q

SUPER HOT on bone scan

A

pagets

126
Q

what stains for vimentin and Epithelial membrane antigen

A

Synovial cell sarcoma - often shows calcificiations - tx with wide resection and chemor or rads

127
Q

tx of NOF

A

obs ; curettage and bone graft if symptomatic;

128
Q

what percent of mets have drug pumps

A

50%; (25% in primary cancers)

129
Q

when to worry about osteochondroma

A

if > 2cm then needs more surveilance; or if growing after skeletal maturity

130
Q

lipoblastoma

A

infants get this; respond with marginal excision;

131
Q

NOF histo

A

swirling fibrous tissue with giant cells;

132
Q

café au lait seen in NF

A

coast of california

133
Q

tx of pagets

A

supportive tx; if symptomatic then bisphos (or calcitonin if they cant tolerate)

134
Q

Chondrosarcoma survival rates

A

Grade I 90%; II 60-70%; III 30-50%; dedifferentiated is 10%

135
Q

Ewings stain

A

no effect on survivall, only local recurrence

136
Q

gene in pagets

A

5q35 QTER gene

137
Q

mets of synovial sarc go to

A

lung 30-60%

138
Q

osteoid osteoma on bone scan

A

HOT

139
Q

PNST (neurilemoma) histo

A

verocay bodies (nuclear pallisading); Antoni A(cellular); Antoni B(myxoid)

140
Q

synovial sarcoma survival linked to

A

SIZE

141
Q

Cold on Bone Scan

A

Renal, Thyroid, Multiple Myeloma (and eosinophillic granuloma)

142
Q

work up Multiple myeloma

A

labs+ BM aspirate for marow involvment; >10% plasma cells (minor criteria); or > 30% major criteria, - normal is 2-3% plasma cells

143
Q

Mafucci syndrome

A

multiple enchondroma and hemangioma

144
Q

management of asymptomatic enchondroma

A

obs

145
Q

UBC in calc

A

will shrink to skeletal maturity and eventually go away

146
Q

general tx of high grade soft tissue sarcoma

A

Rads + Surg. Chemo only if systemic or unresectable

147
Q

tx of fracture from UBC

A

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

148
Q

Clear Cell Sarcoma HPI

A

20-40yo; in feet; dark lesion a/w tendons; dorsum of foot; poor prognosis

149
Q

clear cell histo

A

clear cytoplasm, stains for S100; HMB45; c12:22 translocation; spindle cells throughout

150
Q

Multiple myeloma HPI

A

pain is a LATE finding; needs a skeletal survey;

151
Q

tx of osteosarc is

A

chemo, wide excision, chemo and recon

152
Q

fibrous dysplasia in front of tibia is

A

osteofibrous dysplasia - observation only; on histo look for osteoblast rimming

153
Q

how to distinguish a malignant chondroid lesion

A

larger (>8cm); older age, central skeleton, night pain, local recurrence, hot on bone scan, axial skeleton (vs appendicular)

154
Q

Leiomyosarc histo

A

cigar nuclei; lots of pink cytoplasm; stain for desmin/actin and vimentin (sarcoma marker)

155
Q

what tumors express desmin

A

rhabdomyosarcoma

156
Q

recurrance for chondrosarc related to

A

increased telomerase activtity

157
Q

poor prognostic factors in Ewings

A

spine, pelvic tumors, large size, < 90% necrosis on chemo, high LDH, p53 mutation

158
Q

CD 99

A

Ewings /PNET - can use in synovial sarcoma too

159
Q

CD1a

A

Eosinophilic granuloma; histiocytic lesions

160
Q

fibrous dysplasia heals with

A

fibrous dysplasia

161
Q

tx of liposarcoma

A

rads and surg

162
Q

multiple NOF part of what syndrome

A

Jafffe capanacci - includes vision problems, Mental retardation; and café-au-lait

163
Q

PVNS

A

hemosiderin, INTRA articular; - the non-synovial version of this is giant cell tumor of tendon sheath

164
Q

myxoid liposarc gene

A

t12:16

165
Q

Leiomyosarc found in

A

retroperitoneum usually but can be in extremities - arise from Smooth muscle

166
Q

tx of leiomyosarc

A

wide resection and XRT - poor prognosis

167
Q

osteoid osteoma histo

A

big ring of bone with tiny cells and mucoid in the middle

168
Q

tumoral calcinosis

A

rare; in African american women; a/w metabolic defecfts, collagen disorders, and trauma - look for calcific debris on xray that’s lobular

169
Q

tx of chordoma

A

wide resection; if unresectable or margins are questionable then XRT

170
Q

radiation effect on soft tissue sarcoma

A

no effect on survivall, only local recurrence

171
Q

multiple myeloma stain

A

CD38

172
Q

Epitheloid sarcoma HPI

A

young adults; hand or forearm - nodular on histology; lots of eosinophillic cytoplasm; can spread via lymph

173
Q

multiple fibrous dysplasia and endocrin issues

A

Albright’s syndrome

174
Q

low grade chondrosarc adjuvant

A

phenol or argon beam with grafting if its a low grade chondrosarcoma

175
Q

BCR ABL is

A

philadelphia c=some in CML

176
Q

rhabdomyosarc HPI

A

most common under age 15; embryonal is most common overall and in kids; alveolar is most common in extremities;

177
Q

osteosarc limb salvage vs amp

A

equivalent outcomes so choose limb salvage

178
Q

Ewings gene

A

EWS-FL1

179
Q

tx for myxoid liposarc

A

wide excision and XRT

180
Q

Bright T1

A

Fat, Marrow, Proteinaceious Fluid, Gadolinium, Met Hg, Melanin,

181
Q

tx of desmoid tumor

A

usually chemo and nsaids for large lesions; if surg then WIDE margins bc of reccurrence issues +/- XRT

182
Q

what is side effect of doxorubicin

A

cardiac toxicity; dexrazoxane used to mediate the toxicity

183
Q

why cant you use bone scan for stress fracture

A

wont be hot until at least 72 hrs after

184
Q

melorheostosis

A

waxed bony run off - HOT on bone scan

185
Q

Ewings workup requires

A

bone scan, bM biopsy, and Chest CT

186
Q

stains for rhabdomyosarc

A

MyoD1; Myoglobin, myosin, desmin, and vimentin

187
Q

tx of pagets sarcoma

A

chemo; wide resect; recon

188
Q

4 types of rhabdomyosarcoma

A

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

189
Q

in patient with pagets what are you at risk of

A

osteosarcoma

190
Q

histo of pagets looks like

A

osteoblastoma

191
Q

soft tissue calcifications

A

synovial sarcoma - look for CENTRAL Ca for malignant stuff

192
Q

prognosis of synovial sarcoma linked to

A

size <5cm all survived; 5-10cm 75%; and >10 cm 20% survival

193
Q

myoD1 and myogenin Myf 4 stain found in

A

skeletal muscle

194
Q

most common soft tissue sarcoma in adults overall

A

High Grade Spindle Cell Sarcoma

195
Q

Neuroblastoma

A

INFANTS - look for large adrenal mass on CT scan or bone scan; stain + for NB84; true rossets (circular cell pattern)

196
Q

what med can you give with GCT

A

denosumab - antibody to RANKL

197
Q

Eosinophillic Granuloma

A

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

198
Q

histo of talangiectatic osteoS

A

dilated blood filled vascular space lined by malignant osteoblasts

199
Q

3 stages of GCT

A

capanacci: 1 in bone; II expands bone with rim; III into soft tissue

200
Q

chordoma looks like _ on histology

A

liposarcoma - massive bubble cells

201
Q

synovial chromatosis can progress to

A

synovial chondrosarcoma

202
Q

CD 31

A

most specific endothelial maerk - stains normal endothelail cells

203
Q

enchondroma

A

Long, oval, with well defined margin - look for thinned cortex that has expanded; small round nuclei with chondrocytes

204
Q

what cx is Rads and Chemo RESISTENT

A

classic intramedullary CHONDROSARCOMA; tx is wide surgical excision

205
Q

Chondromyxoid sarcoma gene

A

EWS-CHN 9;22

206
Q

desmoid tumor (fibromatosis)

A

extra abdominal lesion; shouilder is most common site;

207
Q

stains for synovial sarc

A

vimentin, keratin, and Epith Membrane antigen

208
Q

café au lait seen in in Albrights

A

coast of maine (ragged)

209
Q

tx of epitheloid sarc

A

usually wide resection but may need amputation as this travels up the arm

210
Q

tx of osteopetrosis

A

Bm transplant

211
Q

EMA (epitheloid membrane antigen)

A

epitheloid sarcoma, synovial sarcoma,

212
Q

signet ring cell seen with

A

liposarcoma

213
Q

tx of PVNS

A

simple excision vs complete synovectomy; the extra articular analogy is GCT of tendon sheath

214
Q

Malignant PNST

A

50% start from PNST; tx w/ resection and XRT - prognosis is < 20%

215
Q

histo of High grade spindle cell sarcoma

A

pleomorphic and storiform - herringbone pattern ‘ tx is surg + XRT; chemo if systemic; poor prognosis

216
Q

xray findings of chondrosarcoma

A

look for scalloping of the cortex with periosteal reaction

217
Q

Gauchers disease bone deformity

A

ALSO erlenmeyer flask deformity bc of gluccocerebrosides in marrow; tx with enzyme replacement (glucocerebrosidase)

218
Q

tx of fibrous dysplasia

A

obs for asx lesions; otherwise curettage and bone graft

219
Q

most common soft tissue sarcoma in foot

A

Synovial cell sarcoma - often shows calcificiations

220
Q

NF -1 a/w with which tumor

A

MPNST

221
Q

simply bone cyst

A

90% are prox femur or prox humerus; usuall tx with obs and then deal with cyst; look for fallen frag of cortex into cyst;

222
Q

Tx of MM

A

chemo and XRT +/- bisphos to preent new lytic lesions

223
Q

EXT 1 vs EXT 2

A

EXT 1 has higher rate of sarcoma transformation; more limb malalignment, shorter segments and more pelvic-flat bone involvment

224
Q

overal MOST common soft tisuse sarcoma

A

LIPOSARCOMA

225
Q

giant cell tumor on MRI

A

dark on T1

226
Q

MHE with EXT 1 VS 2

A

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

227
Q

Blue Cell Tumors

A

lymphoma, ewings, Rhabdo, neuroblastoma, myeloma

228
Q

osteoblastoma histo

A

wavy thick bone with cells in b/w

229
Q

osteosarc survival without chemo

A

17%

230
Q

periosteal chondroma HPI and xr

A

painful in prox humerus - seen as small lytic lesion INSIDE the cortex - projects into soft tissue space