Building upon our understanding of its molecular pathophysiology, in the HLRCC population, the combination of EGFR and VEGF inhibition (erlotinib/bevacizumab) has been shown to be effective, with response rates of up to 50% seen in the AVATAR study [74] and a separate retrospective analysis [75], highlighting this as a valid therapeutic option in this subgroup. Here, EGFR is linked to hereditary leiomyomatosis and renal cell cancer.