Table 2: Summary of studies using irinotecan-based therapy for patients with relapsed/refractory Ewing sarcoma.

Author [Ref] 𝑁 IRN dose and scheduleRoute of IRN administrationTEM doseOther drugsResponse rate (complete + partial)Comments

Bisogno
et al. [38]
1320 mg/m2/d × 5 × 2IV38%Study limited to soft tissue PNET patients
Cosetti
et al. [37]
320 mg/m2/d × 5 × 2IV0
Bomgaars et al. [29]1650 mg/m2/d × 5IV0
Casey
et al. [36]
2010 mg/m2/d × 5 × 2IV100 mg/m2/d × 563%Median TTP  =  8.3 months
Wagner
et al. [35]
1410–20 mg/m2/d × 5 × 2IV100 mg/m2/d × 529%50% of patients received at least 6 courses before progression
Anderson et al. [44]2510 mg/m2/d × 5 × 2IV100 mg/m2/d × 560%Median TTP  =  5.5 months
Wagner
et al. [31]
535–90 mg/m2/dayPO100–150 mg/m2/d × 5VCR40%*Phase I trial using different doses and schedules
McNall-Knapp et al. [46]115 mg/m2/d × 5 × 2IV100 mg/m2/d × 5VCR100%  ( 𝑛 = 1 )

*Two of five patients had initial responses, but came off study prior to repeat imaging VCR, vincristine; TEM, temozolomide; IRN, irinotecan; TTP, time to progression.