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

Table 3

Targeted agents for treatment of MPNST: previous and ongoing clinical trials.

Drug	Target	Phase		Population	Outcome	Results	Ref.
Erlotinib	EGFR	II	24	≥18 y; Refractory	Response; WHO [101]	19/20 pts.; PD at 2 months; 1 SD	[96]
Sorafenib	C-Raf; B-Raf VEGFR2 C-Kit PDGFR	II	12	≥18 y; Refractory	Response; RECIST [102]	No responses; median PFS 1.7 months	[97]
Imatinib	C-Kit PDGFR VEGFR	II	7	>10 y; Refractory	Response; RECIST [102]	No responses; 1 SD	[98]
Dasatinib	C-Kit SRC	II	14	≥13 y; Refractory	Response; CHOI [103]	No response or SD	[99]
Alisertib	AURKA	II	10	≥18 y; Refractory	Response; RECIST [102]	No response; PFS 13 weeks	[100]
Bevacizumab/RAD001	Angiogenesis/mTOR	II	—	≥18 y; Refractory	Response; WHO [101]	Currently ongoing	—
Ganetespib/Sirolimus	Hsp90; mTOR	I/II	—	≥16 y; Refractory	Response; WHO [101]	Currently ongoing	—