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Table 4 Literature data of chemotherapy agents and TT for advanced TT

From: Antiangiogenic effects in patients with progressive desmoplastic small round cell tumor: data from the French national registry dedicated to the use of off-labeled targeted therapy in sarcoma (OUTC’s)

Chemotherapy agent Patients number Best response PFS References
Vinorelbine-Cyclophosphamide 2 2 PR 4 and 15 months Ferrari et al. [18]
Irinotecan 8 4 SD, 4 PD Unknown Bisogno et al. [19]
Trabectedine 2 2 SD 4 months Frezza et al. [21]
TT agent
 Imatinib 7 1 PR, 4 PD, 2 NA 1 month Chao et al. [22]
 Sunitinib 8 2 PR, 3 SD, 3 PD 2.6 months Italiano et al. [23]
 Pazopanibc 9 2 PR, 5 SD, 2 PD, CBRa 78% 9.2 months Frezza et al. [24]
 Temsirolimus 1 SD 10 months Thijs et al. [33]
 Ganitumab 16 1 PR, 10 SD, 4 PD, CBRb 25% 19 months Tap et al. [30]
  1. PR partial response, SD stable disease, PD progressive disease, NA non assessable, CBR clinical benefit rate (PR + SD >12 weeksa, 24 weeksb)
  2. cRetrospective analysis from data comprising three DSRCT patients treated within the EORTC phase II study 62043, three in the EORTC phase III study 62072 (PALETTE), along with three patients treated in the UK on the subsequent pazopanib named patient program