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Table 2 Treatment response and duration

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)

Patient First treatment Surgery resection quality Radiotherapy PFS 1 Relapses treatments/PFS Lines of treatment before TT TT type PFS with TT (months)
1 MAIDa-ASCTb R2 _ 12 months Gemcitabine-cisplatine/2,5 months 2 Sunitinib 2
2 LV5Fu-Ciplatine _ _ 8 months HIPECc-FOLFIRId/17 months FOLFIRI/3 months Holoxan-etoposide/3 months Adriamycine-cyclophosphamide/2 months 5 Sorafenib 3.5
3 MAIe R1 Yes 39 months Gemcitabine-docetaxel/4 months Adriamycine-holoxan/3 months Cisplatine-irinotecan/6 months Trabectedine/9 months 5 Sorafenib 4
4 Adriamycine-etposide-Cisplatine-Cyclophosphamide + VAIf R2 _ 44 months Carboplatine-etoposide/13 months 2 Sunitinib 5.5
5 Cyclophosphamide-etoposide-carboplatine R1 Yes 47 months Etoposide-carboplatine-busulfan-thiotepa/12 months Temozolamide/5 months TEMIRIg/4 months 4 Bevacizumab 2
6 MAI _ _ 6 months VACh/6 months 2 Ridaforolimus PFS 23 months Sunitinib 4 OS 38 months
7j Bevacizumab-IVADoi R1 Yes 7 months Navelbine-cyclophosphamide/10 months 2 Dalotuzumab PFS 2 months Sunitinib 3
8 Adriamycine-ifosfamide-etoposide _ _ 6 months Cyclophosphamide/3 months Trabectedine/4 months 3 Sunitinib 2 OS 19 months
9 MAI _ _ 7 months VAC/3 months 2 Sunitinib 2
  1. aMesna-Adriblastine-Ifosfamide-Dacarbazine
  2. bAutologous stem cell transplantation
  3. cHyperthermic intraperitoneal chemotherapy
  4. d5 Fluorouracile-Oxaliplatine-Irinotecan
  5. eMesna-Adriblastine-Ifosfamide
  6. fVincristine-Actinomycine D-Ifosfamide
  7. gTemozolamide-Irinotecan
  8. hVincristine-Actinomycine D-Cyclophosphamide
  9. iIfosfamide-Vincristine-Actinomycine D-Doxorubicine
  10. jThis patient had TT bevacizumab in first line treatment as part of BERNIE study in combination with IVADo polychemotherapy. The second relapse was treated with dalotuzumab, he received temodal-irinotecan for third relapse (PFS 8 months) and he finally had sunitinib for the fourth relapse