Table 2: Studies consisting of patients with mixture of relapsed and primary metastatic Ewing’s sarcoma (outcomes of patients with relapsed Ewing’s sarcoma reported separately).

StudyAverage age (years)Total number of ptsES number ptsNumber of HDCT patients (ES rel)ChemotherapyResponse (number of pts)HDCT (mg/m2; mg/kg)Source of stem cellEFS/RFSOS

Ekert et al. 1984, retrospective [20]1222Total (4)
Relapsed (2)
2V
A
Dacarbazine
CR (1)V (1.5 mg/m2)
Cytoxan (1200 mg/m2)
Imidazole (250 mg/m2)
A (20 mg/m2)
BSCRel-1 yr RFS-0%Rel-2 yr OS 50% (HDCT)

Pole et al. 1984, retrospective [21]1518Total (8)
Relapsed (6)
5V
Cytoxan
A
Actinomycin
RP.SAMelphalan (120–210 mg/m2)BSCRel-mEFS (HDCT)-3 monthsNL

Burdach et al. 1993, retrospective [22]1617Total (17)
Relapsed (10)
10Cytoxan
I
VP16
CR (6)
PR (4)
Melphalan (120–180 mg/m2/dose)
Etoposide (60 mg/kg)
TBI
PBSC
BSC
Rel-4 yr EFS 50%
All pts: 6 yr-45% (HDCT)
Rel-4 yr OS 50%

Parentesis et al. 1999, retrospective [23]5–2824Total (9)
Relapsed (5)
5 (3 pts. in CR)NoneCR (3)
PR (2)
Melphalan (50 mg/m2)
Thiotepa (300 mg/m2)
Busulfan (1 mg/kg)
Etoposide (1800 mg/m2)
PBSC
BSC
Rel-3 yr. EFS 66 % (CR) (HDCT)Rel-3 yr OS 66% (CR)(HDCT)

Jahnukainen et al. 2015, retrospective [24]NR24Total (9)
Relapsed ES (1)
Relapsed EFT (3)
1Cytoxan
D
DactinomycinI
CR (1)Thiotepa (900 mg/m2)BSCNLRel (EFT, ) 2 yr OS = 100%
Rel (ES, )-8.5 years (HDCT)

Seo et al. 2013, retrospective [25]13.49Total (9)
Relapsed (5)
5V
I
D
CR (4)
SD (1)
Cytoxan (1500 mg/m2/day)
Melphalan (60 mg/m2/day)
PBSCRel-2 yr EFS 40%
All pts: 2 yr EFS 45% (HDCT)
Rel-1 yr OS 60%/2 yr OS 40%
All pts: 2 yr OS 45% (HDCT)

Rosenthal et al. 2008, prospective single arm [26]1620Total (20)
Relapsed (14)
14V
A
ICE
CR (9)
PR (2)
SD (1)
PD (2)
C1: melphalan/busulfan
C2: melphalan/carboplatin or busulfan or others
Melphalan (140 mg/dose)
BSCRel-1 yr. EFS-36%
All pts: 1 yr EFS 45%/3 yr EFS 47% (HDCT)
Rel-1 yr. OS-50%
All pts: 1 yr OS 60%/3 yr OS 45% (HDCT)

Frohlich et al. 1999, retrospective [27]NL131Total (131)
Relapsed (52)
52NLNLMelphalan (30 mg/m2/day, d1-4)
Etoposide (1800 mg/m2, d1)
TBI
PBSC
BSC
Rel-HDCT: 4 yr EFS 17%
Without HDCT: 4 yr EFS 2% (historical control patients)
NL

Juergens et al. 2009, retrospective [28]NL32Total (32)
Relapsed (11)
11NLNLMelphalan (140 mg/m2)
Treosulfan (36 g/m2)
NLNLRel-3 yr OS 70% (HDCT)

HDCT: high-dose chemotherapy, TBI: total body irradiation, CR: complete response, PR: partial response, PBSC: peripheral blood stem cell, BSC: bone marrow stem cell, SCT: stem cell transplant, EFS: event-free survival, OS: overall survival, NL: not listed, M: melphalan, V: vincristine, E: etoposide, C: carboplatin, B: busulfan, P: procarbazine, I: ifosphamide, A: adriamycin, D: doxorubicin, ES: Ewing’s sarcoma, rel: relapsed, RP.SA: response present, specifics unavailable, RFS: relapse-free survival, EFT: Ewing’s family of tumors, and ES rel: patients with relapsed ES that got HDCT.