Table 1.

The inhibitors targeting the VHL/HIF axis.

	Drugs or compounds	Targets or mechanisms	Clinical trials for PPGLs (www.clinicaltrials.gov)
Tyrosine kinase inhibitors	Sunitinib	Targeting VEGFR-1,2, PDGFR-β,RET, FGFR	NCT01371201, NCT00843037
	Sorafenib	Targeting RAF kinase, c-KIT, FLT-3, RET, VEGFR1-3, and PDGFR-β	None
	Cabozantinib	Targeting VEGFR, MET, RET	NCT02592356, NCT04400474, NCT02302833
	Axitinib	Targeting VEGFR	NCT01967576, NCT03839498
	Lenvatinib	Targeting VEGFR, FGFR, RET, c-Kit, PDGFα	NCT03008369, NCT02592356
	Pazopanib	Targeting VEGFR1-3, PDGFR-α,β, c-Kit	NCT01340794
Non-selective HIFs inhibitors	17-AAG	Promoting protein degradation	None
	17-DMAG	Promoting protein degradation	None
	Vorinostat	Promoting protein degradation	None
	Topotecan	Inhibiting translation and transcription activity	None
	Acriflavine	Inhibiting heterodimerization	None
	2-Methoxyestradiol	Inhibiting nuclear translocation and transcriptional activity	None
	YC-1	Inhibiting protein accumulation and transcription activity	None
	Doxorubicin/daunorubicin	Inhibiting DNA binding	NCT00002764, NCT00002608, NCT00002641
HIF-1α inhibitors	PX-478	Inhibiting mRNA expression and translation	None
	EZN-2208	Inhibiting mRNA expression	None
	Chetomin	Disrupting binding to p300	None
	Echinomycin	Inhibiting DNA binding	None
	KC7F2	Inhibiting protein synthesis	None
	Glyceollins	Inhibiting protein synthesis and stability	None
	Bisphenol A	Promoting protein degradation	None
	LW6	Promoting protein degradation	None
	PX-12	Promoting protein degradation	None
	Cryptotanshinone	Blocking nuclear translocation	None
	cyclo-CLLFVY	Inhibiting heterodimerization	None
	Indenopyrazole 21	Inhibiting transcriptional activity	None
	EZN-2968	Inhibiting mRNA expression and translation	None
HIF-2α inhibitors	PT2385	Inhibiting heterodimerization	None
	PT2399	Inhibiting heterodimerization	None
	PT2977	Inhibiting heterodimerization	None