Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatrics > Ewings Sarcoma > Flashcards

Ewings Sarcoma Flashcards

1
Q

Ewings Sarcoma originates from

A

Neuroectoderm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Most commonly seen in

A

Lower extremities > Upper extremities
Axial skeleton 25%
Pelvis 25%
Femur 20%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Pathology Patho gnomic findings

A

Small round blue cell tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what are all the small round blue cell tumors

A

LEARN & RM (~ learn and remember)

L: Lymphoma
E: Ewings sarcoma
A: ALL
R: Rhabdomyosarcoma
N: Neuroblastoma/Neuroepithelioma

R: Retinoblastoma
M: Medulloblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Median Age, sex and race

A

10-15 yrs
Caucasians
Males

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Ewings family tumors

A

Ewings sarcoma - 87%
Ewings extraosseous sarcoma - 8%
Peripheral PNET - 5%
Askins tumor - PNET of chest wall: Sarcoma fo the ribs -> pleural extension

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Staging

A

NO formal staging
75% localised
25% have Mets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Common sites of mets

A

Lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

H & P

A

Don’t forget to do Neuro and MSK examination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Labs

A

CBC, CMP, LDH, ESR, UA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Imaging

A

X-ray: Lytic, sclerotic, moth eaten, onion skin, codman triangle
MRI with contrast: needed for diagnosis
CT Chest
PET-CT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

X-Ray findings

A

Onion skin appearance = Periosteal stranding.
Periosteum is displaced by underlying tumor.

Seen in the Diaphysis of the bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

DD for periosteal stranding/thickening

A

Ewings Sarcoma
Osteosarcoma
Fractures
Osgood-Schlatter disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Diagnostic study, Pathology form where

A

Bone marrow biopsy - Bilateral and from the tumor
CT guided core needle biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Common translocations

A

EWSR 1 gene translocation

t(11:22) - 85%
t(21:22) - 5-10%
(q24:q12)
(q21:Q12)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Treatment paradigm

A

Induction Chemotherapy x 6 cycles followed by
Local therapy @ week 12 either with SX or XRT followed by
Chemotherapy x 11 cycles (upto 48 weeks total) followed by
Consolidation of met sites if any

17
Q

Chemotherapy

A

Dose Dense VDC q 2 weeks
V: Vincristine
D: Adriamycin
C: Cyclophosphamide

18
Q

Response rate to induction chemotherapy

A

90%

19
Q

Adults chemo

A

q 3 weeks as they do not tolerate well

20
Q

SX

A

Preferred over XRT in children due to risk of secondary malignancy from XRT
Better local control

21
Q

XRT

A

Pre -Op if you think there will be close margins
Adjuvant XRT given along with chemotherapy week 14.

3600- Pre-OP
4500 - Definitive RT
5040- PORT

22
Q

Volumes

A

GTV 1: Pre chemo T2flare with modified margins
CTV 1: GTV1 + 1cms = 4500cGy/25fx (for extra osseous & LN can go upto 5040 and no need GTV2)
GTV 2: Post tissue soft tissue tumor + Pre chemo bone volume
CTV 2: GTV2 + 1cms = 5580cGy/33fx

23
Q

Metastatic disease consolidation

A

Given after all the chemotherapy
SBRT: 40Gy/5fx to gross disease and 35Gy to 3mm expansion
Lung Mets: Whole lung irradiation 15Gy/10fx with boost to gross disease with 45Gy
Ascites: Whole abdomen radiation: 24Gy/16fx

24
Q

PORT

A

50% risk of local failure.
PORT is given when
- Close margin <1cm for bone. <0.5cm for soft tissue
- Intraoperative tumor spill
- <90-95% necrosis

25
Q

5-yr OS for extremity localized disease

A

80%

26
Q

5-yr OS for Pelvic localized disease

A

60%

27
Q

5-yr OS for Lung mets

A

50%

28
Q

5-yr OS for bone marrow mets

A

30%

29
Q

Local failures occur in the first

A

2 years

30
Q

Staining

A

PAS
MIC2 glycoprotein
Vimentin

Negative for
S100
NSE

31
Q

Clinical Symptoms

A

Pain >90%
Swelling/mass
Limitation of motion
pathological fractures
fever

32
Q

Poor Prognostic factors

A

MASS LDH

M: Male
A: Age>17
S: Site, Size>8cm
S: Stage

LDH: High
P53 expression
INK4A deletion

33
Q

What markers help differentiate between EWS and other small round cell tumors

A

Increase in MYC
B2 microglobulin
HBA-71
Vimentin

34
Q

What markers help differentiate between EWS and PNET

A

PNET = S-100 and NSE +ve
EWS = Homer Wright rosettes

Pediatrics (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions