Role of High-Dose Chemotherapy and Autologous Hematopoietic Cell Transplantation for Children and Young Adults with Relapsed Ewing’s Sarcoma: A Systematic Review

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).

Study	Average age (years)	Total number of pts	ES number pts	Number of HDCT patients (ES rel)	Chemotherapy	Response (number of pts)	HDCT (mg/m^2; mg/kg)	Source of stem cell	EFS/RFS	OS
Ekert et al. 1984, retrospective [20]	12	22	Total (4); Relapsed (2)	2	V; A; Dacarbazine	CR (1)	V (1.5 mg/m^2); Cytoxan (1200 mg/m^2); Imidazole (250 mg/m^2); A (20 mg/m^2)	BSC	Rel-1 yr RFS-0%	Rel-2 yr OS 50% (HDCT)
Pole et al. 1984, retrospective [21]	15	18	Total (8); Relapsed (6)	5	V; Cytoxan; A; Actinomycin	RP.SA	Melphalan (120–210 mg/m^2)	BSC	Rel-mEFS (HDCT)-3 months	NL
Burdach et al. 1993, retrospective [22]	16	17	Total (17); Relapsed (10)	10	Cytoxan; I; VP16	CR (6); PR (4)	Melphalan (120–180 mg/m^2/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]	5–28	24	Total (9); Relapsed (5)	5 (3 pts. in CR)	None	CR (3); PR (2)	Melphalan (50 mg/m^2); Thiotepa (300 mg/m^2); Busulfan (1 mg/kg); Etoposide (1800 mg/m^2)	PBSC; BSC	Rel-3 yr. EFS 66 % (CR) (HDCT)	Rel-3 yr OS 66% (CR)(HDCT)
Jahnukainen et al. 2015, retrospective [24]	NR	24	Total (9); Relapsed ES (1); Relapsed EFT (3)	1	Cytoxan; D; DactinomycinI	CR (1)	Thiotepa (900 mg/m^2)	BSC	NL	Rel (EFT, ) 2 yr OS = 100%; Rel (ES, )-8.5 years (HDCT)
Seo et al. 2013, retrospective [25]	13.4	9	Total (9); Relapsed (5)	5	V; I; D	CR (4); SD (1)	Cytoxan (1500 mg/m^2/day); Melphalan (60 mg/m^2/day)	PBSC	Rel-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]	16	20	Total (20); Relapsed (14)	14	V; A; ICE	CR (9); PR (2); SD (1); PD (2)	C1: melphalan/busulfan; C2: melphalan/carboplatin or busulfan or others; Melphalan (140 mg/dose)	BSC	Rel-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]	NL	131	Total (131); Relapsed (52)	52	NL	NL	Melphalan (30 mg/m^2/day, d1-4); Etoposide (1800 mg/m^2, 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]	NL	32	Total (32); Relapsed (11)	11	NL	NL	Melphalan (140 mg/m^2); Treosulfan (36 g/m^2)	NL	NL	Rel-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.