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H/O RC > STS > Flashcards

STS Flashcards

1
Q

STS epi

A

RMS 50%

Fibrihistiocytic
unspecific
synovial
(myo)fibroblastic
MPNST
leiomyosarcoma
liposarcoma

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2
Q

infant STS top 3

A

RMS
infantile fibrosarcoma
malignant rhabdoid tumor

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3
Q

adolescent STS top 3

A

RMS
synovial sarcoma
MPNST

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4
Q

RMS IHC, genetics

A

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%)

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5
Q

RMS genetic predisposition

A

NF1
LFS
Gorlin
Noonan
Costello
BWS

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6
Q

RMS Oberlin RF

A

<1 or 10+yo
unfavourable primary site
3+ met sites
bone/BM involvement

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7
Q

RMS location frequency

A

GU 23%
other
Extremity 20%
H+N 36%
- parameningeal 15%
- orbit 10%
- other H+N 10%

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8
Q

RMS favourable sites

A

biliary
H+N
orbit
GU (nonprostate/bladder)

(BH OG good)

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9
Q

RMS mets

A

15-25%

locoregional LN
lung
BM
bone

prostate: lung

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10
Q

RMS tx

A

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
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11
Q

RMS ISRG Stage

A

I: fav site, no mets
II: unfav site, <5cm, no mets
III: unfav site, >5cm or <5cm with nodes, no mets
IV: distant mets

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12
Q

RMS ISRG Group

A

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

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13
Q

RMS risk stratification

A

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

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14
Q

RMS ix

A

CT/MR primary site
CT chest
PET

mets: b/l BMA/B
parameningeal: LP
extremity of paratesticular: LN biopsy

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15
Q

RMS chemo backbone

A

VAC (VCR, dactino, cyclo)
+ VI for IR
+VDC/IE for HR

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16
Q

RMS px

A

LR 80-90%
IR 70%
HR ARMS 10% ERMS 60%

17
Q

RMS relapse tx and px

A

palliative
resect if possible
neoadj chemo
- VAC/VI
- VIDE (VCR, ifos, etop, doxo)

6mPFS 65%, 5yPFS 17%
OS 20-30%

18
Q

ERMS/ARMS likely by site
- GU
- extrem
- orbit

A

ERMS: GU, orbit
ARMS: extremity

19
Q

NRSTS top 2 chemo

A

ifos
doxo

20
Q

NRSTS poor chemo response

A

MPNST
alveolar soft part sarcoma
epithelioid sarcoma
fibrohistiocytic
(myo)fibroblastic

21
Q

NRSTS likely to spread to LN

A

clear cell sarcoma
epithelioid sarcoma (15%)
less common: desmoplastic small round cell tumor, synovial sarcoma

22
Q

NRSTS associated with FAP

A

desmoid fibromatosisd

23
Q

desmoid fibromatosis disease association

A

FAP

24
Q

infantile fibrosarcoma genetic

A

t(12;15)
ETV6-NTRK3

larotrectinib

25
Q

Genetic predisposition:
- leiomyosarcoma
- MPNST
- malignant rhabdoid
- desmoid fibromatosis
- dermatofibrosarcoma protuberans

A
  • leiomyosarcoma: RB germline
  • MPNST: NF1
  • malignant rhabdoid: SMARCB1
  • desmoid fibromatosis: FAP
  • dermatofibrosarcoma protuberans: ADA SCID
26
Q

NRSTS associated with:
- RB germline
- NF1
- SMARCB1
- FAP
- ADA SCID

A
  • leiomyosarcoma: RB germline
  • MPNST: NF1
  • malignant rhabdoid: SMARCB1
  • desmoid fibromatosis: FAP
  • dermatofibrosarcoma protuberans: ADA SCID
27
Q

NRSTS risk stratification

A

LR: localized, gross resection
IR
- high grade and >5cm
- unresectable
HR: metastatic

28
Q

NRSTS px

A

LR 5yEFS 90% OS 96%
IR 5yEFS 65% OS 80%
HR 5yEFS 20% OS 35%

29
Q

NRSTS tx principles

A

complete resection most important

neoadj chemo/RT if unresectable or morbid resection

IR/HR:
- chemo: ifos/doxo
- adj RT

30
Q

synovial sarcoma epi, path, tx, px

A

extrem, mets 7% (lung most common)

SS18 fusions

excision, RT, chemo if unresectable/mets/>5cm
- pazopanib

5yEFS 80% OS 90%

H/O RC (31 decks)

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