STS Flashcards
STS epi
RMS 50%
Fibrihistiocytic
unspecific
synovial
(myo)fibroblastic
MPNST
leiomyosarcoma
liposarcoma
infant STS top 3
RMS
infantile fibrosarcoma
malignant rhabdoid tumor
adolescent STS top 3
RMS
synovial sarcoma
MPNST
RMS IHC, genetics
muscle specific actin
myosin
myogenin
MYOD1
desmin
myoglobin
ERMS: fusion neg; LOH11p15, RAS
(- Embryonal nEg)
ARMS: FOXO1 fusion, ALK copy number, n-MYC amplification, CDK4 amplification
- t(2;13) PAX3-FOXO1 (60%)
- t(1;13) PAX7-FOXO1 (20%)
RMS genetic predisposition
NF1
LFS
Gorlin
Noonan
Costello
BWS
RMS Oberlin RF
<1 or 10+yo
unfavourable primary site
3+ met sites
bone/BM involvement
RMS location frequency
GU 23%
other
Extremity 20%
H+N 36%
- parameningeal 15%
- orbit 10%
- other H+N 10%
RMS favourable sites
biliary
H+N
orbit
GU (nonprostate/bladder)
(BH OG good)
RMS mets
15-25%
locoregional LN
lung
BM
bone
prostate: lung
RMS tx
resection (goal GTR)
chemo
RT
- IR/HR
- local control if unresectable (parameningeal, orbital, prostate, vaginal)
- 50.4 Gy gross residual
- 36 Gy micro residual
- 41.4 Gy nodal
- 45 Gy orbital
RMS ISRG Stage
I: fav site, no mets
II: unfav site, <5cm, no mets
III: unfav site, >5cm or <5cm with nodes, no mets
IV: distant mets
RMS ISRG Group
I: localized, complete resection, neg margins
- a) no extension, b) extension
II: GTR with regional spread
- a) + margin, b) LN, c) both
III: gross residual
- a) post bx, b) post 50+% resection
IV: distant mets
RMS risk stratification
LR
- group I-III stage I-2
IR
- group I-III FP
- group I-II stage 3 FN
- group III FN
- group IV stage IV FN <10yo
HR
- group IV stage IV FN >10yo
- group IV stage IV FP
RMS ix
CT/MR primary site
CT chest
PET
mets: b/l BMA/B
parameningeal: LP
extremity of paratesticular: LN biopsy
RMS chemo backbone
VAC (VCR, dactino, cyclo)
+ VI for IR
+VDC/IE for HR
RMS px
LR 80-90%
IR 70%
HR ARMS 10% ERMS 60%
RMS relapse tx and px
palliative
resect if possible
neoadj chemo
- VAC/VI
- VIDE (VCR, ifos, etop, doxo)
6mPFS 65%, 5yPFS 17%
OS 20-30%
ERMS/ARMS likely by site
- GU
- extrem
- orbit
ERMS: GU, orbit
ARMS: extremity
NRSTS top 2 chemo
ifos
doxo
NRSTS poor chemo response
MPNST
alveolar soft part sarcoma
epithelioid sarcoma
fibrohistiocytic
(myo)fibroblastic
NRSTS likely to spread to LN
clear cell sarcoma
epithelioid sarcoma (15%)
less common: desmoplastic small round cell tumor, synovial sarcoma
NRSTS associated with FAP
desmoid fibromatosisd
desmoid fibromatosis disease association
FAP
infantile fibrosarcoma genetic
t(12;15)
ETV6-NTRK3
larotrectinib
Genetic predisposition:
- leiomyosarcoma
- MPNST
- malignant rhabdoid
- desmoid fibromatosis
- dermatofibrosarcoma protuberans
- leiomyosarcoma: RB germline
- MPNST: NF1
- malignant rhabdoid: SMARCB1
- desmoid fibromatosis: FAP
- dermatofibrosarcoma protuberans: ADA SCID
NRSTS associated with:
- RB germline
- NF1
- SMARCB1
- FAP
- ADA SCID
- leiomyosarcoma: RB germline
- MPNST: NF1
- malignant rhabdoid: SMARCB1
- desmoid fibromatosis: FAP
- dermatofibrosarcoma protuberans: ADA SCID
NRSTS risk stratification
LR: localized, gross resection
IR
- high grade and >5cm
- unresectable
HR: metastatic
NRSTS px
LR 5yEFS 90% OS 96%
IR 5yEFS 65% OS 80%
HR 5yEFS 20% OS 35%
NRSTS tx principles
complete resection most important
neoadj chemo/RT if unresectable or morbid resection
IR/HR:
- chemo: ifos/doxo
- adj RT
synovial sarcoma epi, path, tx, px
extrem, mets 7% (lung most common)
SS18 fusions
excision, RT, chemo if unresectable/mets/>5cm
- pazopanib
5yEFS 80% OS 90%
