Table 1.

Summary of xenograft and GEM mouse models of OS and ES discussed in review.

Malignancy	Mouse model	Significance to preclinical studies of drug development	Mouse strain or genetic alteration	Cell line	Refs.
OS	Subcutaneous	Comparative screening of multiple tumors of different karyotypes	CB17SC-M scid−/−	Patient-derived (OS-1, OS-2, OS-17, OS-9, OS-33, OS-31, OS-29)	[22]
	Orthotopic	Evaluation of spontaneous metastasis	C.B-17	143B	[27]
			BALB/c	K7M2	[30]
			C3H	DLM8	[29]
			NOD/SCID	143B	[28]
		Modification of targets by gene silencing or overexpression	Unknown	SaOS-2	[26]
	Metastatic	Test metastatic xenografts generated from metastatic cell lines	BALB/c	Zos-M	[31]
			Nu/nu	KRIB	[32]
			NOD/SCID	143B	[33]
	Resistant	Study of mechanisms of resistance and resistance -modifying agents	C3H/Sed	MOS/ADR1	[25]
			C3H/Sed	MOS/ADR2
	Spontaneous	Evaluation of naturally developing tumors	Fischer		[40]
	GEM	Study tumors in native environment and with intact immune system	Osx-Cre+p53fl/flpRbfl/fl		[38]
			Osx-Rbc/c;p53c/c		[39]
ES	Subcutaneous	Screening of tumors expressing EWS-FLI1	BALB/cJHanHsd-SCID	Mouse MPC	[50]
		Modification of EWS-FLI1 expression by gene silencing	BALB/c nu/nu	SK-N-MC	[51]
		Comparative screening of multiple tumors	CB17SC-M scid−/−	Patient-derived (EW-5, EW-8), SK-NEP-1, TC-71	[22]
	GEM	Study tumors in native environment and with intact immune system	Prx1-Cre EWS-FLI1		[61]

MPC: Mesenchymal progenitor cell.