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Haem/Onc > Sarcomas, ASPHO > Flashcards

Sarcomas, ASPHO Flashcards

1
Q

most imp somatic mutations in RMS?

A

PAX-FOX0O1 fusions in alv. RMS t(2;13)

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2
Q

most common PAX_FOXO1 in RMS?

A

PAX3-FOXO1 (2;13) =60% >PAX7-FOXO1 (t;13)=20%

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3
Q

PAX-FOXO1 mut is a ___ in tumorigenesis

A

driver

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4
Q

give 2 genetic changes seen in fusion neg RMS

A

aneuploidy
activation muts of RAS pathway (FGF4, NRAS, KRAS, etc muts)
p53 muts= unfav
myoD muts= unfav
LOH or Loss of Imprinting at 11p15.5
NCOA2, VGLL2 rearrangements= fav in infants with spindle cell RMS

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5
Q

Give 5 germline muts associted with RMS

A 
Li-Fraumeni=p53
BWS=IGF2
Costello=HRAS
Noonan= RAS-MAPK path
NF1= NF1
Gorlin= PTCH1
PBB= Dicer 1
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6
Q

bimodal peak for general sarcomas adn soft tissue sarcomas?

A

infancy and teenage years

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7
Q

presentation of RMS?

A

mass
+/- pain
+/- distrubance in function like CN palasy or bladder dysfunction

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8
Q

most common site of RMS?

A

head and neck>GU>extrem

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9
Q

favourable H&N site for RMS

A

orbit, non-parameningeal

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10
Q

parameningeal site= unfavourable in rhabdo…waht does this include?

A

base of skull, including infratemporal fossa, paranasal sinuses, parapharyngeal area

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11
Q

favourable GU site for RMS?

A

paratsticular, vag, uterine

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12
Q

unfavoruable GU site for rhabdo?

A

bladder, prostate

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13
Q

lymphatic spread of RMS seen where?

A

paratesticular, extremity tumours

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14
Q

CNS ext seen where in RMS?

A

50% parameningeal

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15
Q

hematogenous mets go where in RMS?

A

lung, bone, BM, liver

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16
Q

stage RMS how?

A
  • MRI or Ct of priamry site adn regional nodal basin
  • CT chest
  • PET scan or bone scan
  • bilateral BMA/bx
  • CSF cytomology if parameningeal/paraspinal
  • LN for paratestictular at least 10 years of age or extremity
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17
Q

WHich RMS pts do NOT need bone scan, BMA/bx, possible chest CT?

A

ERMS, T1, N0 disease

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18
Q

what’s the name of the MRS staging system?

A

IRS

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19
Q

describe stage 1 or IRS staging for RMS

A
  • favourable site: orbit, non-parameningieal head and neck, non-bladder/prosatate GU, biliary tract
  • T1= confined to site of origin or T2= extension and/or fixation to surroundign tissue
  • size: any
  • any regional LN status
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20
Q

describe stage 2 for RMS staging

A
  • unfav site: parameningeal, bladder/prostate, extremity, other
  • T1 or T2 (same)
  • size <5 cm
  • regional lymph nodes not present or unknwon
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21
Q

describe stage 3 for RMS

A
  • unfav site
  • T1 or T2 (same)
  • size: <5 cm if just regional LNs involved, vs. >5 cm for LNs not involved/regional involvement/unknown
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22
Q

describe stage 4 RMS

A
  • all sites with distant mets
  • T1 or T2 (same)
  • any size
  • regional LNs involved or not
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23
Q

what does RMS grouping tell you?

A

IRS grouping tells you extent of tumour remaining after SURGERY, before any other tx

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24
Q

Describe RMS group 1

A

completely resected lcoal disease

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25
Q

describe group 2 in RMS

A

gross total resection with evdience of regional spread…
A): microscopic residual diseae
b) reigonal nodes involved AND completely researcted
C) regional nodes invovled and grossly resected but have microscopic residual disease and histo involvement of the resected regional nodes

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26
Q

describe group 3 rmS

A

incomplete resection with gross residual disease

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27
Q

describe gropu 4 RMS

A

distant mets

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28
Q

what’s very prog in RMS?

A

staging= extent of disease

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29
Q

3 yr FFS in stages 1-4 RMS?

A

86%
80%
68%
25%

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30
Q

how does group impact prog?

A

Groups1-3 have better 5-yr FFS and 5-yr OS (73% and 84%) than group 4 (30% and 42%)…..also 3-yr FFS gets worse with higher group: 83%->86%->73%->25%

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31
Q

RMS histo subtypes in fusion neg?

A

spiondle cell, botryoid, embryonal

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32
Q

what if you are fusion negative but alveolar RMS?

A

better prog, simialr to embryonal histo

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33
Q

describe RMS risk gruops (COG)

A

low risk has a 5 yr FFS of at lesat 85%…drops to 50-75% for intermed and 20% or less for high…to be low risk, must be stage 1-2 with group I-II diseaes, embryonal OR stage 1, group III ORBIT, embryonal….high risk: stage 4, group 4, at lesat 10 yrs of age with emrbryonal or stage 4+ group 4 wtih alv. RMS (fusion pos)…everything else= intermed

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34
Q

primary site in RMS = prognostic. best site? worst site? order?

A

best= orbit/head and neck
worst= extremity
order. orbit/head and neck>GU non-bladder/prostate> then GU ballder/prostate, parameningeal>other>extremity

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35
Q

other than primary site, genetic mutations, staging, group, what else is prog in RMS?

A
  • mets…mets to lung better than to bone/bone marrow.
  • and # of met sites…2 or less is better than 3 or more!
  • recurrence…~20-25% 3-5 yr OS
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36
Q

if relapsed RMS, waht are good RFs?

A

botryoid histology

ERMS that was stage 1 or group 1 at initial dx with local or regional recurrence adn not previously tx’ed iwth cyclophos

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37
Q

what are 3 main types of childhood RMS

A

embryonal, alveolar, spindle cell

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38
Q

ERMS and spindel cell RMS fall in teh category of ___ ___ tumours; what else is in this category?

A

spindle cell; synovial sarcoma

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39
Q

how can you differentiate synovial sarcoma from RMS (ERMS or spindle cell RMS)?

A

ERMS and spindle cell RMS: pos for MyoD, myogenin, Desmin, muscle specific actin…but synovial: cytokeratin, EMA, Bcl2

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40
Q

ARMS falls in teh category of what tumours?

A

Small round blue cell tumour

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41
Q

small round blue cell tumour pos for NSE, synaptophysin, TH, PHOX2b?

A

NBL

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42
Q

small round blue cell tumour + for CD99, vimentin, +/-NSE, synaptophysin?

A

EWS/PNET

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43
Q

SRBC tumour + for myoD, myogenin, desmin, muscle specific actin?

A

ALVEOLAR RMS

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44
Q

SRBCT pos for LCA< CD3, CD20, TdT?

A

lymphoma

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45
Q

most common type of RMS?

A

embryonal, ~2/3

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46
Q

ERMS has includes a ___ pattern

A

botryoid

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47
Q

ERMS associated with a ___ age, ___ site, ___ disease, ___ prog

A

younger; favourable; localized; favourable

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48
Q

spindle cell RMS: associated with ___ or ___ rearrangements in infants and a ___ prog

A

NCOA2; VGLL2; favourable

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49
Q

what % of alv RMS is fusion neg?

A

20%

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50
Q

RMS tx involves? broadly

A

surgery, radiotherpay, chemo

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51
Q

tx in RMS based on?

A

risk groups

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52
Q

RMS risk groups based on what things? 4

A

stage
group
histo/fusion status
age (soemtimes)

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53
Q

surgical approach in RMS?

A

excise pirmary tumour upfront whenever possible wihtout –> major functional or consmetic defs…sometimes wait to do surgery if think may spare radiation

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54
Q

which RMS sites reqwuire surg assessmetn of LNs?

A

paratesticular (retroperitoneal for boys 10 yrs+), extremity

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55
Q

who doesn’t need rads in RMS?

A

group 1 ERMS/spindle cell RMS

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56
Q

do radiation when in RMS?

A

usually begins during week 3-15 of therpay

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57
Q

dose of rads in RMS

A

36-50.4 Gy

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58
Q

Standard= interm risk chemo in RMS?

A

VAC x 45 weeks = vcr, dactinomycin, cyclophos

59
Q

low risk chemo in RMS?

A

VA…or shorter duration with VA + lower CPM dose

60
Q

high risk chemo in RMS?

A

no standards…can add doxo, epto, ifos, irinotecan

61
Q

image how often in RMS?

A

q3 months

62
Q

response at 12 weeks: predictive of survival in RMS?

A

no

63
Q

aucte tox in RMS?

A

heme tox, infection, VOD with dactinomyicn and cyclophos

64
Q

late effects of RMS?

A

infert due to alk agents 7.5-8 mg/m2 esp in boys (standard dose and higher)…secondary maligs due to alk agents/radiation/CPS…bone growth/arrest/muscle atrophy due to radiation

65
Q

non rhabdo soft tissue: tend to occur where? spread wehre?

A

extremities, trunk….lungs, bone, LNs

66
Q

general work up for non rhabdo soft tissue tumours?

A

MR/Ct of primary, Ct chest, PET scan, nodal eval for select tumorus

67
Q

non rhabdo soft tissue sarcomas: prog depends on?

A

stage, group (extent of resection), tumouor size (< or >5 cm), grade (mitotic rate), tumour type, age

68
Q

ilst 3 non-rhabdo soft tissue sarcomas that are reposneive to chemo

A

synovial, undifferentatied, infantile fibrosarcoma

69
Q

most common non-rhabdo soft tissue sarc?

A

synovial

70
Q

transloc in synovial sarc?

A

t(X;18) SYT-SSX

71
Q

tranlsoc seen in infantile fibrosarcoma?

A

t(12;15)= ETV-NTRK3 fusion…most common STS in children <1 yrs…give VA, VAC, or larotectinib if not resected

72
Q

3 non rhabdo soft tissue sarcs that are poorly responsive to chemo

A

MPNST, leiomyosarcoma, alveolar soft part sarcoma, epithelioid sarcoma

73
Q

MPSNT asscoiated with what CPS?

A

NF1

74
Q

sarcoma associated with immunosuppression and HIV?

A

leiomyosarcoma

75
Q

fusion seen in alv soft part sarcoma?

A

t(X;17) = ASPSCR1-TFE3 fusion

76
Q

non rhabdo STS: tx philopshy?

A

surgery + radiotherpay….+/- chemo

77
Q

general tx approach in nrSTS?

A

based on extent of resection…group 1: observe unless grade 3 or large, then do RT (and maybe chemo= doxo + ifos)….group 2: RT alone unless grade 3 or large, then consdier chemo (Doxo + ifos)…group 3-4: RT, delayed surgery, consider chemo

78
Q

what’s being investigated in NRSTS?

A

pazopanib= tki

79
Q

give 2 cytogen changes seen in osteosarcoma

A

chromothripsis

supernumary ring chromosone 12q13-15

80
Q

3 molecular abnoramliteis seen in osteosarcoma

A

Rb, p53, RECQL4 helicase mutations (germline)

81
Q

peak age for osteo?

A

~15

82
Q

3 rfs for osteo?

A

ionizing rad, hereditary rb, li fraumeni

83
Q

presentation of oseto?

A

pain +/- mass invovlign metasphyses of long bone

84
Q

3 most common primary sites in osteo?

A

distal femur> prox tibia>prox humerus

85
Q

what is a skip lesion?

A

pattern of spread of osteo; can be several cm from primary, in the same bone…occurs in 20% of osteo

86
Q

how does osteo spread?

A

skip lesions adn hematgenous spread

87
Q

sites of osteo mets? (2)

A

lung>bone

88
Q

what % of osteo has mets at dx?

A

15-20%

89
Q

stage osteo how?

A

plain films of primary
MRI of primary
CT chest
PET scan or bone scan

90
Q

stage assginemnt in osteo based on waht two things?

A

localized or not

resectable or not

91
Q

localized osteo 3 yrs EFS?

A

65-70%

92
Q

5 yrs EFS in met osteo?

A

<30%

93
Q

in osteo, bettter to have unilateral pulm mets or bone mets?

A

unilateral pulm mets

94
Q

describe role of necrosis in osteo

A

if at lesat 90% necrosis at 10 week of pre-op chemo, better 3-yr EFS…80% vs 60%

95
Q

types of histo that are favorable in osteo?

A

parosteal, fibroblastic, telangiectatic

96
Q

how does lcoation and size play a role in osteo prog?

A

extremity better than axial; 8 cm or less better

97
Q

relapsed osteo don’t do well. RFs for shorter survival after realpse?

A

age >10
mets at dx
lung and bone recurrence
time to relapse<2 yrs

98
Q

pathognomic features on histo for osteo?

A

osteoid-producing malig cdells

99
Q

2 major histo subtypes of osteo?

A

central (medullary) and surface tumours

100
Q 
for the following types of OS, indicate if central=medullary or surface
1-small round cell
2-low grade central
3-parosteal
4-telangiectatatic
5-conventional
6-periosteal
7-high grade surface
A 
1-central
2-central
3-surface
4-central
5-central
6-surface
7-surface
101
Q

3 features of osteo on x-ray?

A
  • mixed osteoblatsic+ oteolytic features
  • cortical destruction adn expanstion into soft tissue
  • periosteal reaction, eg: codman triagle, sunburst pattern
102
Q

ewings is more ___ than osteo

A

osteolytic

103
Q

5 ddx for osteosarcoma?

A 
ewing sarcoma
mets
LCH
oseomyelitis
osteoblastoma
aneurysmal bone cyst
fibrous dysplasia
104
Q

tx philosophy for osteo?

A

SURGICAL resection of all gross tumour adn chemo

105
Q

timing of surgery in osteosarcoma?

A

after 10 weeks of neoadj chemo

106
Q

advanrages of neoadj chemo in osteo?

A
  • allows starting therapy more rapidly
  • may faciliate limb salvage surgery
  • provides histo response
107
Q

chemo in osteo?

A

HD-Mtx, doxo, cisplatin= MAP

108
Q

what type of osteo doesn’t need chemo?

A

PAROSTEAL

109
Q

what’s teh diff between parosteal and periosteal osteosarcom?

A

parosteal: tends to occur in distal femur, is low grade, reqwuires resection only!
periosteal: tends to occur at prox tibia, is intemdiate grade, tx with resection +/-chemo

110
Q

3 things you may see on imaging that indicate osteo improving?

A
  • increased ossification
  • sclerosis or calcification or border
  • reduction in soft tissue mass
111
Q

late effects in osteo?

A

cardiomyopathy (athracyclin)…in >20%

cisplatin-indcued ototox

112
Q

most common fusion in EWS?

A

EWSR-FLI1 fusion = t(11;22), 85%!

113
Q

2nd most common EWS fusion?and another?

A

21;22…2;22

114
Q

detect EWS transloc how?

A

RT PCR or FISH

115
Q

EWS: more common in black or white pts?

A

white

116
Q

presenation of EWS?

A

pain +/- palp mass

117
Q

EWS: what part of bone involved?

A

diaphyses and central axis

118
Q

most common site in EWS (3)

A

pelvis>femur>rib

119
Q

top 3 met sites in EWS?

A

lung>bone>BM

120
Q

what % of EWS have mets?

A

20%…same as osteo

121
Q

EWS staging how?

A 
plain films
MRI or CT of primary
CT chest
PET or bone scan
bilateral BMA/bx
122
Q

assign stage how in EWS?

A

local vs mets

123
Q

most imp prog featuers in EWS?

A

stage

124
Q

in terms of prog, worst primary site in EWS?

A

pelvis

125
Q

describe how tumour size and age are import in EWS prog

A

better if younger

better if <8 cm

126
Q

2 stains used in EWS?

A

CD99, vimentin

127
Q

EWS can have differentaition or not…if does have diff, see what? (3)

A

rosettes
NSE
synaptophysin

128
Q

what features of EWS are seen on x-ray?

A

lytic lesion with a wide zone of transition and periosteal reaction…see aggressive periosteal reactions like codman triangle, sunburst pattern or onion skin pattern

129
Q

ddx for EWS on x-ray?

A

osteosarcoma, mets, LCH, osteomyelitis

130
Q

tx philosophy for EWS?

A

surg, radiation, neoadj chemo, (SCT)…use surg when possible

131
Q

tx in EWS based on what 2 things?

A

stage, site

132
Q

rad dose in EWS?

A

36-50.4 Gy

133
Q

chemo in EWS?

A

VDC IE= vcr, doxo, cyclo, ifos, etop

134
Q

other htan VDC IE, what are other active agents in EWS?

A

topotecan, iriontecan, temozolomide

135
Q

role of SCT in EWS?

A

possibly for high risk localized EWS

136
Q

late effects in EWS?

A

Radiation: –> SMNs (carcinomas, sarcomas, 10%); path fractures/limb length discrepancy/femoral head necrosis..
Chemo: leukemias, cardiomyopathy from athracyclin, ifosfamide-induced nephrotoxicity = dose-dep proximal tubular damage

137
Q

BRIEFLY describe bio of RMS vs NRSTS vs OS vs EWS

A 
ERMS: aneuploidy, point mutations
ARMS: PAX-FOXO1 fusions
NRSTS: many tumour types
OS: chrom instability
EWS: TET-ETS fusion: EWSR1-FL1
138
Q

most common sites for RMS/NRSTS/OS/EWS?

A

RMS: head, GU tract
NRSTS: extrems, trunk
OS: metaphyseal, distal femur adn prox tibia
EWS: diaphyseal or soft tissue, pelvis and central axis most common

139
Q

risk based on what for RMS/NRSTS/OS/EWS?

A

RMS: stage, group, fusion status, histo
NRSTS: stage, group, tumour size, grade, histo
OS: stage, resectability
EWS: stage, site

140
Q

histo of RMS/OS/EWS?

A 
ERMS: spindle cell tumour
ARMS: SRBCT
RMS in general: myogenin, desmin +
OS: osteoid-producing malig cells
EWS: SRBCT, CD99+, may show neural diff
141
Q

tx in RMS?

A

upfront surgery +/- delayed RT/surgery…systemic= VAC +/- VI

142
Q

tx in NRSTS?

A

local: surgery (may be dleayed) +/- RT (may be pre-op)…systemic= +/- Doxo, ifos depending on chemo sens

143
Q

tx in OS?

A 
local= delaeyd surgery
syst= MAP
144
Q

EWS tx?

A 
local= delayed surgery adn/or RT
syst= VDC/IE

Haem/Onc (30 decks)

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