Table 2.
Published studies of conventional and novel agents for patients with relapsed classical Ewing sarcoma (EwS).
| Reference | Year of Publication | Agents Evaluated | Key Findings |
|---|---|---|---|
| Trials of conventional chemotherapy for relapse | |||
| [236] | 2009 | High-dose ifosfamide | High-dose ifosfamide is active in relapsed EwS previously treated with standard-dose ifosfamide |
| [242] | 2006 | Topotecan/cyclophosphamide | 33% of patients with PR and 27% with SD |
| [243] | 2000 | Topotecan/high-dose cyclophosphamide | Responses seen in patients with EwS |
| [244] | 2004 | Irinotecan/temozolomide | Responses seen in patients with EwS |
| [245] | 2009 | Irinotecan/temozolomide | 63% ORR |
| [246] | 2012 | Gemcitabine/docetaxel | Limited activity seen in patients with EwS |
| [238] | 2020 | High-dose ifosfamide vs. topotecan/cyclophosphamide vs. irinotecan/temozolomide vs. gemcitabine/docetaxel |
High-dose ifosfamide and topotecan/cyclophosphamide arms superior to irinotecan/temozolomide and gemcitabine/docetaxel. Enrollment is ongoing. |
| Trials of targeted therapies for relapse | |||
| [250] | 2017 | Regorafenib (REGO) | 10% objective response rate and a median PFS of 3.6 months |
| [251] | 2020 | Cabozantinib (CABONE) | 26% ORR |
| [252] | 2010 | Figitumumab | 2/16 patients with PR |
| [253] | 2011 | Figitumumab | 14% of patients with PR |
| [254] | 2011 | R1507 | 10% ORR |
| [255] | 2012 | Ganitumab | 6% ORR |
| [256] | 2014 | Olaparib | No patients with objective response |
| [257] | 2020 | Talazoparib/temozolomide | No patients with objective response, 15 with SD |
| [258] | 2020 | Talazoparib/irinotecan +/− temozolomide | 73% of patients had a clinical response (1 CR; 4 PR; 11 SD) |
| [259] | 2020 | TK216 | Two CR have been reported Enrollment is ongoing |
| [260] | 2020 | Seclidemstat | Enrollment is ongoing |
SD, stable disease; ORR, objective response rate; PFS, progression-free survival; PR, partial response; CR, complete response.