Interestingly, vascular cells in BHD-associated HOCT demonstrated increased oxidative phosphorylation without an upregulated HIF-Vascular endothelial growth factor (VEGF) signature compared to clear cell renal cell carcinomas, suggesting that receptor tyrosine kinase inhibitors targeting tumor angiogenesis may not be effective in BHD-associated kidney cancer and a totally different therapeutical approach may be required for targeting vascular cells in BHD-associated kidney cancer. This evidence concerns the gene VEGFA and Birt-Hogg-Dube syndrome.