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SADL Oncology > Sarcoma > Flashcards

Flashcards in Sarcoma Deck (40):
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)]

31

Most common sites for Osteo vs EWS

Osteo: distal femur, prox tibia, prox humerus

EWS: pelvis, femur, rib

32

what location is most common for RMS?

head and neck is most common
followed by GU tract

33

how do you stage a RMS (from a classification standpoint?)

start with pre-treatment TNM to get the STAGE
then do the postoperative grouping to get GROUP

COG risk group is determined into low, intermediate or high risk based on the stage, group and histology

34

what group of RMS do not require radiation?

Group I ERMS

35

which chemotherapy agent in RMS treatment is a radiosensitizer?

Dactinomycin

36

what are the 3 most common locations for OS?

metaphysis of
1-distal femur
2-proximal tibia
3-proximal humerus

37

what are the theoretical advantages for neoadjuvant chemotherapy in OS?

1. decrease in tumor-related edema and shrinkage of the tumour at the primary site to allow for better surgery
2. initial chemo directed toward micromets (we know are present in 80-90% patients)
3. assessment of the sensitivity of the primary tumour to chemo which has prognostic significance

38

what are the preliminary results of AOST0331?

this was a combined study with EURAMOS
those with good response (>90% necrosis) were randomized to MAP vs MAP plus pegylated interferon alpha 2B
those with poor response randomized to MAP or MAPIE

neither experimental arms showed a survival advantage

39

for relapsed OS what salvage regimens can be used

high dose ifosfamide with etoposide
Gemcitabine and docetaxel

should do full resection if able
radiation can be used for palliation

40

RF for NRSTS

p53
NF-1 (MPNST)
FAP (desmoid-type fibromatosis)
SMARCB1 (extra-renal rhabdoid tumor)
RB1 (leiomyomatosis)
Werner's