Table 1: Studies consisting of patients with only relapsed Ewing’s sarcoma.

StudyMedian age (years)Number of ptsType of disease (number of pts)Type of relapse (number of pts)ChemotherapyHDCT (mg/m2; mg/kg)Number of HDCT ptsSource of stem cellResponse (number of pts)EFSOS

Rasper et al. 2014, registry study [14]NL239Local (42)
Metastatic (197)
Local (42)
Distant (142)
Combined (30)
T/C
I/Te
If/E/carboplatin
C1: busulfan/melphalan (NL)
C2: treosulfan/melphalan (NL)
73
C1 = 15
C2 = 38
COR = 20 (ONR)
NLPrior to HDCT:
C1: CR (5)
PR (2)
C2: CR (19)
PR (8)
2 yr HDCT: 44–47%
5 yr HDCT: 20–24%
2 yr HDCT: 53–66%
5 yr HDCT: 40–42%

McTiernan et al. 2006, retrospective [15]1933Local (22)
Metastatic (11)
Local (11)
Distant (18)
Combined (4)
Ca
E
C
A
I
D
V
Busulfan/melphalan (600 mg/m2/140 mg/m2) (22 pts)
Melphalan/etoposide (130 mg/m2/60 mg/m2) (7 pts)
TBI
Melphalan/cytoxan (16 mg/kg/60 mg/kg) (1 pts)
Melphalan (150 mg/m2) (3 pts)
33PBSC
BSC
Prior to HDCT:
CR (14)
PR (10)
Post HDCT:
CR (21)
PR (2)
2 yr EFS 42.5%
5 yr EFS 38.5% (HDCT)
2 yr OS 50.7%
5 yr OS 42.8% (HDCT)

Barker et al. 2005, retrospective [16]13.555Local (30)
Metastatic (25)
Local (6)
Distant (39)
Combined (10)
VDC-IE
VACIME
VDC-A
VDC-A-M
Busulfan/melphalan/THIO (NL)13PBSCPrior to HDCT
CR/PR (27)
5 yr HDCT: 61% (HDCT)5 yr HDCT: 77% (HDCT)

Ferrari et al. 2015, retrospective [17]NL107NLLocal (11)
Distant (96)
High I
VDCAIE
Busulfan/melphalan (4 mg/kg and 140 mg/m2)20PBSCNLNL5 yr OS HDCT: 50%

Palmerini et al. 2009, retrospective [18]1772NLLocal (11)NLBusulfan/melphalan (4 mg/kg and 140 mg/m2)
Palliative
24NLNLNL3 yr. HDCT: 33%

Shankar et al. 2003, retrospective [19]1464Local (64)Local (11)Ca/E
C/E/Ca
C/E
Melphalan (NL)
TBI
7PBSC
BSC
NLmRFS = 16 monthsmOS = 49 months (HDCT)

Bacci et al. 2003, retrospective [3]18195NLLocal (57)
Distant (138)
NoneMelphalan (NL)
Busulfan (NL)
35
ONR = 2
PBSC
BSC
NL5 yr with HDCT: 21.2%mOS with HDCT: 27.1 m

TBI: total body irradiation, HDCT: high-dose chemotherapy, SCT: stem cell transplant, CR: complete response, PR: partial response, EFS: event-free survival, OS: overall survival, V: vincristine, E: etoposide, M: methotrexate, A: dactinomycin, If: ifosfamide, C: cyclophosphamide, D: doxorubicin, T: topotecan, I: irinotecan, Te: temozolomide, Ca: carboplatin, NL: not listed, high I: high-dose ifosfamide, mOS: median overall survival, mRFS: median relapse-free survival, COR: consortium of other regimens, and ONR: outcomes not reported.