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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