Table 4. Important study data on the first-line treatment of Ewing’s sarcoma.

Study type	Study arm	Patient number	EFS	p	OS	p	Reference
Maintenance treatment in the standard-risk group
RCT	Total	856	3-Year EFS	n.s.	3-Year OS	n.s.	Le Deley et al. 2014 (e16)
	VAI	425	78.2%		85.5%
	VAC	431	75.4%		85.9%
Maintenance treatment in the standard-risk group
RCT	Total	155	3-Year EFS	0.72	3-Year OS	0.80	Paulussen et al. 2008 (e17)
	VAIA#	76	74%		86%
	VACA#	79	73%		90%
Additional etoposide in the high-risk group
RCT	Total	492	3-Year EFS	0.12	3-Year OS	0.23	Paulussen et al. 2008 (e17)
	VAIA#	240	47%		59%
	EVAIA#	252	52%		62%
Type of induction chemotherapy
RCT	Total	640	Hazard ratio	n.r.	Hazard ratio	n.r.	Brennan et al. 2020 (e27)
	VIDE	320	1.0		1.0
	VDC/IE	320	0.70		0.64
Interval between VDC/IE induction cycles
RCT	Total	568	5-Year EFS	0.048	5-Year OS	0.056	Womer et al. 2012 (e19)
	21 Days	284	65%		77%
	14 Days	284	73%		83%
VAI vs. BuMel in the case of inadequate histological response or initial tumor volume >200 ml
RCT	Total	240	3-Year EFS	0.026*	3-Year OS	0.028*	Whelan et al. 2018 (e22)
	VAI	118	56.7%		72.2%
	BuMel	122	69.0%		78.0%
VAI vs. BuMel bei in the case of lung metastasis
RCT	Total	287	3-Year EFS	0.16*	3-Year OS	0.99*	Dirksen et al. 2019 (e14)
	VAI	143	50.6%		68.0%
	BuMel	144	56.6%		68.2%

*Cox regression analysis of the intention-to-treat cohort

Drugs: A, actinomycin; A#, doxorubici; BuMel, high-dose chemotherapy with busulfan and melphalan; C, cyclophosphamide; D; doxorubicin; E, etoposide

EFS, event-free survival; I, ifosfamide; n.r., not reported; n.s., not significant; OS, overall survival; RCT, randomized controlled trial; V, vincristine, vs., versus