Malignant Peripheral Nerve Sheath Tumors State of the Science: Leveraging Clinical and Biological Insights into Effective Therapies

Table 2

Genetically engineered mouse models (GEMMs).

Tumor suppressors mutated	Method of mutation	Oncogenes overexpressed	Promoter overexpressed	Grade	Latency (months)	Penetrance (%)	REF
Nf1^−/+; Ink4a^−/−; Arf^−/−	Germline^1			High	6.5	26	[79]
Nf1^flox/flox; Pten^flox/flox	Cre (Dhh^+ cells)			High	0.5	92	[80]
Nf1^flox/flox; Pten^flox/+	Cre (Dhh^+ cells)			Low	5.7	42	[80]
Nf1^flox/+; Pten^flox/flox	Cre (Dhh^+ cells)			Low	5.8	82	[80]
Nf1^flox/flox + Nf1; p53shRNA	Cre (Periostin^+ cells)^4			Low	6.1	56	[87]
Nf1^flox/− + Nf1; p53shRNA	Cre (GFAP^+ cells)^4			Low	3.0	73	[87]
Nf1^flox/flox; Ink4a^flox/flox; Arf^flox/flox	Cre (injection)			High	4.1	100	[86]
Nf1^−/+:Trp53^−/+	Germline^2			High	5	81	[77, 78]
Nf1^flox/flox	Cre (Dhh^+ cells)	EGFR	CNP	High	~6	33	[81]
Trp53^−/+	Germline^3	EGFR	CNP	Low-high	9.5	19	[84]
Pten^flox/+	Cre (GFAP^+ cells)	Kras-G12D	lox-STOP-lox^5	High	~6	100	[82]
Pten^flox/flox	Cre (Dhh^+ cells)	EGFR	CNP	High	<1	100	[83]
Trp53^−/+	Germline^3	GGFβ3	P0	Low-high	7.5	95	[57]
NA	NA	GGFβ3	P0	ND	8.7	71	[85]

Spontaneous loss of NF1; ²spontaneous loss of NF1 and p53; ³spontaneous loss of p53; ⁴injection of shRNA into sciatic nerve; ⁵activation by Cre (GFAP⁺ cells).
NA: not applicable; ND: not determined.