Case

Study

Age (y)

Size (cm)

Treatment

Immunohistochemistry

Molecular tests

Follow-up

1*

Vang et al. [4]

28, 15

0.9, 20

S+CT+RT

CD99+ (2 cases)

EWS/FLI1+ (2 cases)

FOD at 18 M

FOD 19 M

2

Scherr et al. [15]

10

6.5

NK

CD99+

No

NA

3

Habib et al. [16]

23

NK

NK

No

No

NA

4

Nirenberg et al. [17]

20

12

S+CT+RT

CD99−

No

DOD 10M

5

Lazure et al. [18]

15

20

S+CT

CDD99+

EWS/FLI1+

FOD 7M

6

Moodley et al. [19]

26

5

CT+RT

No

No

NK

7

Parede et al. [20]

29

5

S+CT+RT

No

No

FOD 8 M

8**

McCluggage et al. [21]

19, 40, 20

4, 3, 6.5

S+CT

S+CT

S of lung M+

CD99+, FLI1−

CD99+ FLI1+

CD99+ FLI1+

RTPCR, FISH−

FISH+

FISH+

NA

FOD 12M DOD (Lung M+)

9*

Cetiner et al. [22]

23, 29

6, 1

S+CT+RT

S+CT

CD99+ (2 cases)

EWS/FLI1+

EWS/FLI1−

FOD 84 M

FOD 61 M

10

Boldorini et al. [23]

52

4

S+CT+RT

CD99+

EWSR1 R +

FOD 12M

11

Halil et al. [24]

14

NK

S+CT+RT

No

No

DOD 9 M (lung M+)

12

Anastasiades et al. [3]

28

3

S+CT+RT

CD99+

No

DOD 12M

13

Kelling et al. [25]

18

NK

S

NK

NK

NK

14

Che et al. [13]

37

NK

S+CT. Lung M+

CD99+, FLI1+

No

AWD 12 M

15*

Xiao et al. [26]

20, 36

NK

No

CD99+ in 2 cases (Biopsy)

No

Bone and lung M+, DOD.

Lung M+, DOD

16

Rekhi et al. [2]

10

8

S+CT

CD99+, Fli1+

ESW/FLI1−/EWSR1 R+

CR. Recurrence with new lung M+. AWD 18M

17

Tunitsky et al. [27]

15

5

S+CT

NK

NK

FOD 20 M

18

Dadhwal et al. [28]

20

20

NK

NK

NK

DOD (metastatic disease)

19

Fong et al. [14]

17

NK

NK

CD99+, FLI1+

EWS/FLI1+

NK

20

Yang et al. [29]

20

NK

No

CD99+

RT-PCR+

DOD (bone and lung M+)

21

Present case

30

16

CT+RT

CD99+

EWSR1 R+

FOD 22 M