Table 2.
Regimen | Outcome Data | Study Design; Number of Patients |
---|---|---|
Doxorubicin, cyclophosphamide, ifosfamide, etoposide | Favorable risk: 3-year FFS 79% Unfavorable risk: 3-year FFS 17% |
RCT [26] Favorable: n = 14 Unfavorable: n = 122 |
Vinorelbine, cyclophosphamide, temsirolimus | 6-month EFS: 69% RR: 47% |
RCT (v. vinorelbine, cyclophosphamide, bevacizumab) [41] n = 87 |
Vinorelbine, oral cyclophosphamide | Median survival: 9 months RR: 36% |
Phase 2 single arm [69] n = 50 |
Vincristine, irinotecan, temozolomide | 3-month PFS: 23% DCR: 27% |
Retrospective analysis [49] n = 19 |
RR: 43% | Retrospective analysis [70] n = 7 |
|
RR: 25% | Retrospective analysis (ARMS) [48] n = 4 |
|
Vincristine, irinotecan | 1-year FFS: 37–38% RR: 26–37% |
Randomized phase 2 window (two schedules) [31] n = 92 |
Cyclophosphamide, topotecan | Median time to progression: 2 months RR: 67% |
Phase 2 single arm [71] n = 15 |
RR: 67% | Retrospective analysis [72] n = 6 |
|
Ifosfamide, carboplatin, etoposide | 2-year OS: 26% 2-year OS for ERMS: 46% RR: 67% |
Retrospective analysis [73] n = 27 |
Topotecan, carboplatin | 5-year PFS: 17% RR: 28% |
Single arm [74] n = 38 |
Gemcitabine, docetaxel | RR: 40% | Retrospective single institution [75] n = 5 |
FFS: failure-free survival; EFS: event-free survival; DCR: disease control rate; RR: response rate; PFS: progression-free survival; RCT: randomized clinical trial.