Table 1 Inclusion and exclusion criteria

• Any histologically and genetically confirmed Ewing sarcoma family of tumours (ESFT) of bone or soft tissue, or round cell sarcomas ‘Ewing’s-like’ but negative for EWSR1 gene rearrangement

(Prior to protocol version 3.0, this read as ‘Histologically confirmed ESFT of bone or soft tissue, and Localised or pulmonary and/or pleural metastatic disease’.)

• Age of more than 2 years and less than 50 years

• Randomisation not more than 45 days after diagnostic biopsy/surgery

• Patient medically fit to receive trial treatment

• No prior treatment other than surgery

• Localised tumour

OR

metastatic disease and/or regional lymph node(s) involvement only at diagnosis and at least partial response of metastases and/or regional lymph node(s)

(Prior to protocol version 3.0, this read as ‘Localised tumour OR Pulmonary and/or pleural metastatic disease only at diagnosis and at least partial response of the lung metastases and no progression of the primary tumour during induction chemotherapy’.)

• Age of more than 5 years

• Consolidation chemotherapy as per protocol intended

• Medically fit to receive zoledronic acid

• Contra-indication to the treatment in randomisation 1 (R1)

• Second malignancy

• Pregnant or breastfeeding women

• History of dental surgery 6 months preceding the start of zoledronic acid or planned dental surgery during treatment or within 6 months after the end of treatment

• History of jaw fracture

• Ewing’s tumour of the maxilla or of the mandible

• Progression of the primary tumour or appearance of new lesions