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Table 4 Sarcoma advisory group guidelines—ewing sarcomas

From: UK guidelines for the management of bone sarcomas

Category

1st line

2nd line

3rd line and other

Localised or metastatic disease with lung or pleural metastases only

VIDE 6 cycles VAC or VAI × 8 cycles [142]

High dose ifosfamide [144] or Ifosfamide and etoposide or cyclophosphamide and topotecan [145] or irinotecan and temozolamide [146] ± High dose chemotherapy (busulphan/lmelphalan or treosulphan/melphalan) with peripheral blood stem cell rescue [147] N.B. Choice of 2nd line therapy will depend on patient/disease specific factors (e.g. if chemo being given with curative intent) and/or patient choice

Cyclophosphamide and topotecan [145] or Irinotecan and temozolamide [146] or gemcitabine and docetaxel [148, 149] or oral etoposide [150] or cisplatin and etoposide

Metastatic disease with bone or bone marrow involvement

VIDE × 6 cycles → VAC or VAI × 8 cycles [142, 143] or VDC/IE interval compressed regimen × 14 cycles [140]

  1. Taken from: London and South East Sarcoma Network (LSESN) Guidelines [98]
  2. V vincristine; I ifosfamide; D doxorubicin; E etoposide; A actinomycin D (dactinomycin); C cyclophosphamide