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SADL Oncology > Sarcoma > Flashcards

Flashcards in Sarcoma Deck (40)
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1

Germline mutations associated with RMS

p53
BWS (11p15 LOH)
Costello (HRAS)
Noonan (Ras-Mapk)
NF-1
Gorlin (PTCH1)
Dicer1

2

Name translocation seen in alveolar RMS

Pax3-FOXO1 t(2;13)

Pax7-FOXO1 t(1;13)

3

RMS immunohistochemical staining to differentiate from other SRBCT

myoD
myogenin
desmin
muscle specific actin

4

Favourable sites for IRS staging

- orbit
- non-parameningeal head and neck
- non bladder/prostate GU
- biliary tract

5

Who can avoid bone marrow?

- non-invasive node-negative tumor
- all NRSTS

6

RMS risk group classification

Low risk:
- all non-met fav-site ERMS
- non-fav site ERMS with CR

Int risk:
- all non-met ARMS
- non-fav site ERMS with PR

High risk:
- met ERMS or ARMS

7

Role of radiation in RMS

- local control for all but grp 1 ERMS
- starts wk 3-15 unless urgent
- 36-50.4 Gy

8

Prognostic factors in RMS

1. Tumor stage
2. Translocation status
3. Extent of metastasis (<2 mets better than more)
4. Site of tumor
5. Age
6. Extent of resection (tumor group)

9

Chemo responsive NRSTS

synovial sarcoma - (X; 18) translocation
undifferentiated sarcoma
infantile fibrosarcoma - (12;15) translocation

10

NRSTS chemo non-responsive

MPNST
Leiomyosarcoma
Aveloar soft part sarcoma
Epitheloid sarcoma

11

Translocations for:
1. Synovial sarcoma
2. Undifferentiated sarcoma
3. Infantile fibrosarcoma
4. Clear cell sarcoma

1. t (X;18) SYT-SSX fusion
2. t(4;19) CIC-DUX4 sometimes
3. t (12;15) ETV6-NTRK3
4. t(12;22) EWSR1-ATF1

12

Prognosis for RMS

Low risk 85%
Int risk 50-75%
High risk <20%

13

Who needs LN exploration in RMS

- clinically suspicious node
- regional node exploration for all extremity lesions
- ipsilateral retroperitoneal LN dissection for boys >10 y.o. with paratesticular tumors

14

Chemo used for NRSTS

Ifos/Doxo

15

ARST 0331 - low risk protocol

- reduce dose of cycle (4.8g/m2)
- shorter length of therapy - 22 instead of 48 wk
Same outcomes as classic VAC

16

ARST 0531 - int. risk protocol

VAC vs VAC/VI - same outcomes

VAC/VI had:
- less hematologic toxicity, less F&N
- lower cyclo dose (8.4 vs 16.8 g/m2)

17

Long term toxicity of therapy

Infertility - related to cyclo and pelvic rads
SMA - 2-3% at 30 yrs related to p53 and rads
Bone growth/atrophy - rads

18

Syndromes associated with NRSTS

1. p53
2. FAP - desmoid fibromatosis
3. Rb - leiomyosarcoma
4. SMARCB1 - extra-renal rhabdoid
5. NF-1 - MPNST

19

Prognostic factors in NRSTS

1. Subtype of tumor
2. Stage (localized vs. metastatic)
3. Group (extent of resection)
4. Size (<5 cm vs. >5cm)
5. Tumor grade (mitotic rate, necrosis, nuclear atypia)

20

NRSTS associated with HIV/immunosuppression

leiomyosarcoma

21

NRSTS likely to spread to LNs

Epithelioid sarcoma and clear cell sarcoma

22

NRSTS most common in < 1yo

Infantile fibrosarcoma and infantile hemangioperiocytoma

23

Osteo:
- cytogenetic abnormality
- molecula abnormalities

cytogenetics: chromothripsis, supernumary ring chromosome (parosteal)

Molecular: Rb, p53, RECQL4 (rothmund thompson syndrome)

24

Prognostic factors in Osteosarcoma

- stage
- number of mets
- extent of resection
- histology (parosteal, telangiectatic, fibroblastic are fav)
- extent of necrosis
- LDH/ALP
- Tumor location (axial unfav)
- size of tumor (9+ cm)

25

Long-term toxicity for osteo

- functional impairment from surgery
- 10% risk of anthracycline related cardiotoxicity
- 10% risk of ototoxicity

26

Most common translocations in Ewing's

t(11;22) EWS-FLI1
t(21;22) EWS-ERG
t(7;22) EWS-ETV6

27

EWS Prognostic factors

Stage - localized vs metastatic
Primary site - extremity better than pelvis
Age
Tumor size (<8cm better)

28

Role of radiation in EWS

- local control for those without good surgical option that preserves function
- in addition to surgery for residual disease

29

Principles of therapy for NRSTS

- Surgery is critical
- everyone gets RT
- role of chemo is controversial - generally reserve for large (>5cm), high-grade or unresectable tumors.

**note if tumor is not chemo-sensitive when deciding.

30

List non-Ewing's sarcoma with EWS fusion transcripts

• Desmoplastic small round cell tumor [t(11;22)(p13;q12), EWS-WT1 fusion]
• Clear cell sarcoma [EWS-ATF1 fusion; t(12;22)(q13;112)]
• Extraskeletal myxoid chondrosarcoma
• Myxoid liposarcoma [EWS-CHOP fusion; t(12;22)(q13;p11)]