Table 2.
Palliative systemic therapy
| Metastatic treatment | Population | Line of treatment | Phase | n (SS) | RR (%) | 3 months PFS (%) | 6 months PFS (%) | PFS (months) | OS (months) | Year of publication |
|---|---|---|---|---|---|---|---|---|---|---|
| Chemotherapy | ||||||||||
| Doxorubicin vs. doxorubicin-ifosfamide [21••] | STS | 1 | III | 454 (64) | 14 vs. 26 | – | – | 4.6 vs. 7.4 | 12.8 vs. 14.3 | 2014 |
| Doxorubicin-palifosfamide vs. doxorubicin[22] | STS | 1 | III | 447 (22) | 28.3 vs. 19.9 | 6.0 vs. 5.2 | 15.9 vs. 16.9 | 2016 | ||
| Doxorubicin-evofosfamide vs. doxorubicin[23] | STS | 1 | III | 640 (33) | 28 vs. 18 | – | – | 6.3 vs. 6.0 | 18.4 vs. 19.0 SS: HR 0.32 (95% CI 0.14–0.73) p = 0·0043 |
2017* |
| Doxorubicin +/− olaratumab [45••] | STS | 1 | Ib-II | 15 (Ib) + 133 (II). SS: 3 | 18 vs. 12 | – | – | 6.6 vs. 4.1 | 26.5 vs. 14.7 (HR 0.47 LMS, 0.56 other) | 2016 |
| Doxorubicin-dacarbazine +/− ifosfamide [55] | STS | 1 | III | 340 (12) | 17 vs. 32 | – | – | 4 vs. 6 | 12 vs. 13 | 1993 |
| Doxorubicin vs. doxorubicin + trabectedin [24] | STS | 1 | II | 113 (7) | 17 vs. 17 | – | – | 5.5 vs. 5.7 | 13.7 vs. 13.3 | 2016 |
| Doxorubicin vs. gemcitabine-docetaxel phase III [25] | STS | 1 | III | 257 (11) | 19 vs. 20 | – | 46.3 vs. 46.4 | 23.3 vs. 23.7 weeks | 76.3 vs. 67.3 weeks | 2017* |
| Ifosfamide [56] | STS | 1, 2 | II | 114 (22) | All: 16 SS: 37 |
Median follow-up 3 years (actuarial estimate): 1 year PFS 15% 1 year OS 50% Median OS 55 weeks |
2000 | |||
| Trabectedin BSC [27•] | Translocation-associated STS | 1–5 | II | 73 (18) | 8 vs. 0% | 70.3 | 44 | 5.6 vs. 0.9 months | Not reached vs. 8.0 (median FU 8.9) | 2015 |
| Eribulin [29] | 4 cohorts STS including SS | 2nd or 3rd | II | 128 (19) | 0 | SS 21.1 | – | – | – | 2011 |
| Gemcitabine-darcabazine vs. dacarbazine[32] | STS | ≥ 2 | II | 109 (11) | 12 vs. 4 | 56 vs. 37 | – | 4.2 vs. 2 | 16.8 vs. 8.2 | 2011 |
| Targeted therapy | ||||||||||
| Doxorubicin +/− olaratumab [45••] | STS | 1 | Ib-II | 15 (Ib) + 133 (II). SS 3 | 18 vs. 12 | – | – | 6.6 vs. 4.1 | 26.5 vs. 14.7 (HR 0.47 LMS, 0.56 other) | 2016 |
| Cixutumumab (anti-IGF-1R antibody) [43] | 5 cohorts STS including SS | All | II | 113 (17) | – | SS 18 | – | SS 6.4 weeks | – | 2013 |
| Cixutumumab (anti-IGF-1R antibody) [42] | Childhood cancer ≤ 31 years | All | II | 114 (11) | All 4% SS 0% |
– | – | – | – | 2014 |
| R1507 (IGF-1R antibody) [44] | STS | All | II | 163 (23) | All 2.5% SS 0% |
– | – | All 5.7 weeks SS 5.7 |
11 months | 2014 |
| Figitumumab (anti-IGF-1R antibody) [41] | Sarcoma | ≥ 2 | I | 29 (5) | All 7% SS 0% |
34% | 28% | – | – | 2010 |
| Imatinib [57] | Sarcoma | All | II | 158 (22) | SS 0% | – | – | SS 1.92 | – | 2009 |
| Regorafenib vs. placebo [38•] | 4 cohorts: SS, LPS, LMS and other | ≥ 2 | II | 182 (27) | 0 | SS: 77 vs. 0% | SS: 38 vs. 0% |
SS: 5.6 vs. 1.0 | SS: no difference | 2016 |
| Pazopanib vs. placebo [35] | STS | 2–5 | III | 372 (44) | 6% | – | – | 4.6 vs. 1.6 | 12.5 vs. 10.7 | 2012 |
| Pazopanib [34] | 4 cohorts STS | All | II | 142 (38) | 14% | SS 49% | – | 161 days | – | 2009 |
| Sorafenib [40] | STS | All | II | 145 (12) | – | – | – | 3.2 | 14.3 | 2009 |
| Gefitinib [58] | HER-1 positive SS | ≥ 2 | II | 46 | 0 | – | 6% | 6 weeks | – | 2008 |
| Panobinostat [59] | STS | ≥ 2 | II | 48 (6) | 0 | 20 | 13.2 | 1.7 | 10.3 | 2013 |
| Immunotherapy | ||||||||||
| Dendritic cell vaccinations [60] | Sarcoma | – | I–II | 37 (2) | 2.9 | – | – | 3 years PFS 2.9% | 3 years OS 44.3% | 2017 |
| Autologous T cells transduced with an NY-ESO-1-reactive T cell receptor after lymphodepleting preparative chemotherapy [54] | NY-ESO-1(+) SS | ≥ 2 | Pilot | 18 | 61 | – | – | – | 3 year OS: 38% 5 year OS: 14% |
2015 |
| Autologous T cells transduced with an NY-ESO-1-reactive T cell receptor after lymphodepleting preparative chemotherapy [53] | NY-ESO-1(+) SS | ≥ 3 | Pilot | 6 | 67 | – | – | – | – | 2011 |
| SYT-SSX-derived peptide vaccines + interferon-α every 14 days 6 times [61] | SS | – | – | 21 | 0% | 33% | – | – | – | 2012 |
SS synovial sarcoma, STS soft tissue sarcoma, OS overall survival, PFS progression free survival, PLS liposarcoma, LMS leiomyosarcoma, RR response rate, FU Follow-Up, HR hazard ratio