Table 2.

Published clinical trials of targeted therapy and PRRT in ACC

Drug	Mechanism	Study phase	Patients	Results	Reference
Figitumumab	IgG2 monoclonal antibody directed at IGF-1R	I	14	ORR 0% SD 43%	[20]
Cixutumumab	IgG1 monoclonal antibody directed at IGF-1R	I/II	12	ORR 16.6% PFS 1.5 m	[21]
Linsitinib	SMI of IGF-1R	III	90	No benefit	[22]
Temsirolimus + cixutumumab	mTOR Inhibitor	I	18	∼ 50% achieved SD	[23]
Sunitinib	Small molecule multi-kinase inhibitor	II	38	SD 13% PFS 2.8 m OS 14–35.5 m	[24]
Sorafenib + paclitaxel	Protein kinase inhibitor	II	10	ORR 0%	[25]
Cabozantinib	Protein kinase inhibitor	Case series	15	ORR 53% PFS 3.0 m OS 9.1 m	[26]
Nilotinib	SM-TKI against PGFR and C-Kit	Pre-clinical/in vitro	0	N/A	[27]
Erlotinib + gemcitabine	EGFR inhibitor	Case series	10	ORR 10%	[28]
Bevacizumab + capecitabine	Humanized anti-VEGF monoclonal antibody	I	10	ORR 0% SD 0% PD 100%	[29]
Axitinib	Selective VEGF-I inhibitor	II	13	PFS 5.5 m OS 13.7 m	[30]
Iodine-131-metomidate	Peptide receptor radionucleotide therapy (PRRT)	Case series	11	ORR 9% SD 45% PD 57%	[31]
Radiolabelled 90Y/177Lu-DOTATOC	Peptide receptor radionucleotide therapy (PRRT)	Case series	2 (selected from 14 based on SSTR2A/SSTR5 avidity)	ORR 100% of treated patients (14% of enrolled patients)	[34]

PFS progression-free survival, OS overall survival, ORR objective response rate, OSD overall survival difference, PD progressive disease, SD stable disease, CR complete response, m months, MMRD mismatch repair deficiency, NPR non-progression rate