First-line Treatment of Ewing Tumours With Primary Extrapulmonary Dissemination in Patients From 2 to 50 Years (CombinaiR3)

"Week 1, 5, 9 and week 13:~* Vincristine, IV, D1, 1.5mg/m²~* Doxorubicine, IV, D1-D2, 37.5mg/m²~* Cyclophosphamide, IV, D1, 1.2g/m²~Week 3, 7, 11 and week 15:~* Ifosfamide, IV, D15 to D19, 1.8g/m²/d~* Etoposide, IV, D15 to D19, 100mg/m²/d"

"Week 1 and week 5:~* Vincristine, IV, D1, 1.5mg/m²~* Doxorubicine, IV, D1-D2, 37.5mg/m²~* Cyclophosphamide, IV, D1, 1.2g/m²~Week 3, and week 7:~* Ifosfamide, IV, D15 to D19, 1.8g/m²/d~* Etoposide, IV, D15 to D19, 100mg/m²/d"

"Week 9, 12, 15 and week 18:~* Temozolomide, PO, D1 to D5, 150mg/m²/d~* Irinotecan, IV, D1 to D5, 50mg/m²/d"

"After induction phase and local treatment (surgery and/or radiotherapy) and before PBSC:~* Busulfan, IV, D-5 to D-2, dosa according to the weight~* Melphalan, IV, D-1, 140 mg/m²~Peripheral Blood Stem Cell infusion:~\- PBSC infusion, D0, at least 3.10\^6 CD34/kg"

"\* 1st year : VC~* Vinblastine, IV, 3mg/m², once a week (except during radiotherapy: 1mg/m², 3 times a week)~* Cyclophosphamide, PO, 25mg/m² continuously"

Surgical excision of whole site of the primary tumour and metastatic sites (outside pulmonary sites) can take place before or after the high dose consolidation chemotherapy. Radiotherapy can be added

Radiotherapy of whole site of the primary tumour and metastatic sites (outside pulmonary sites) can take place before or after the high dose consolidation chemotherapy