Table 1.

Mouse models of human sarcomas

Tumor type	Gene alteration(s)	Agent used	Significance	Proposed karyotype	Reference
Soft Tissue Sarcoma	ND	Rous sarcoma virus	Viral infection influences sarcomagenesis	Unknown	[5]
Osteosarcoma	ND	Radiation	Radiation influences 7 osteosarcoma formation in rabbits	Unknown	[7]
Sporadic/ Varied	p53−/−	None	Loss of p53 results in sarcoma formation	Complex	[40,41]
Sporadic/ Varied	p53R172H mutation	None	Mutations in p53 results in sarcoma formation	Complex	[42,43]
Sporadic/ 31 Varied	Mdm2SNP309 w or w/o p53R172Hmutation	None	Mutations in the p53 pathway result in sarcoma formation	Complex	[31]
Poorly Differentiated Sarcoma	Ews-Fli1Tg w or w/o p53 deletion	Prx-Cre	Ews-Fli1Tgmice fail to induce sarcomagenesis in the absence of p53 loss	Translocation but complex with p53/Rb loss	[44]
Poorly Differentiated Sarcoma/ Osteosarcoma	Ews-Fli1Tg w or w/o Rb and p53 deletions	Prx-Cre	Ews-Fli1Tg;Rb−/− mice fail to induce sarcomagenesis but reduce time of onset in the absence of p53	Translocation but complex with p53/Rb loss	[45]
Osteosarcoma	TaxTg;p19Arf−/−	None	Expression of Tax in the absence of p19Arf results in osteosarcoma formation	Complex	[46]
Osteosarcoma	Rb and p53 deletions	Osterix-Cre	Deletion of p53 and Rb cooperate in the bone leading to osteosarcomagenesis	Complex	[47]
Osteosarcoma/ Leiomyosarcoma	Rb +/- and p107 deletions	None	Rb haploinsufficiency coupled with p107 deletion results in low penetrant Sarcoma formation	Complex	[48]
Developmental defects	Pax-3-Fkhr fusion	None	Pax3-Fkhr fusion product fails to produce sarcomas when expressed from Pax3 promoter	Translocation	[49]
Rhabdomyosarcomas	Pax3-Fkhr transgene w/ and w/o Rb and p53 deletion	Myf6-Cre	Expression of the Pax3-Fkhr transgene requires loss of p53 and Rb for rhabdomyosarcoma formation	Translocation but complex with p53/Rb loss	[50,51]
Rhabdomyosarcomas/ Various sarcomas	Ptch1 heterozygosity w/ and w/o Rb and p53 deletion	Myf6-Cre, Myf-5Cre, or Pax7-CreER	Loss of p53 and Rb in conjunction with Ptch1 haploinsufficiency results in rhabdomyosarcomagenesis	Complex	[52]
Undifferentiated Pleomorphic sarcomas	Mutant K-ras expression and p53 loss	Adenoviral Cre	MutantK-ras expression and p53 loss cooperate in the development of undifferentiated pleomorphic sarcomas	Complex	[53,54]
Pleomorphic Rhabdomyosarcomas	Mutant K-ras expression and p53 loss	Electroporation of Cre into the muscle	MutantK-ras expression and p53 loss cooperate in the development of undifferentiated pleomorphic sarcomas	Complex	[55]
Rhabdomyosarcomas	Mutant K-ras expression and p53 loss or mutation of p53	Ah-Cre	Expression of mutant p53 facilitates a more rapid development of rhabdomyosarcomas than loss of p53 in the mutant K-ras background	Complex	[56]