Table 1: Summary of selected studies using topotecan-based therapy for patients with relapsed/refractory Ewing sarcoma.

Author [Ref] 𝑁 Topotecan dose and scheduleOther drugsResponse Rate (complete + partial)Comments

Hawkins et al. [10]200.3 mg/m2/d for 21-day continuous infusion10%
Blaney et al.
[9]
31–1.3 mg/m2/d for 72-hour continuous infusion4%
Bernstein et al. [12]362 mg/m2/d × 58%Given as upfront window for 6 weeks in newly diagnosed metastatic patients
Bernstein et al. [12]370.75 mg/m2/d × 5CPM 250 mg/m2/d × 557%*Given as upfront window for 6 weeks in newly diagnosed metastatic patients
Saylors et al. [13]170.75 mg/m2/d × 5CPM 250 mg/m2/d × 535%50% of patients received at least 6 courses before progression
Hunold et al. [14]490.75 mg/m2/d × 5CPM 250 mg/m2/d × 533%Additional 26% with stable disease

*In newly diagnosed patients with metastatic disease; CPM, cyclophosphamide.