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 |