The sites of action of pazopanib may in part explain our patient’s improved response to this therapy. In particular, disruption of tyrosine kinase signaling cascades rather than scavenging VEGF-A – the mechanism of bevacizumab – may provide longer-term reduction in tumor burden. Additionally, there may be decreased development of resistance to this therapy. The authors acknowledge that this is a single case report and that no clinical trial has yet been performed to assess the response of ELSTs to pazopanib therapy. Given the rare nature of this condition, a prospective clinical trial is likely not feasible. There remains a dearth of available systemic therapies for vHL syndrome, but as more trials are conducted, the frontiers of treatment options for vHL that may extend to ESLT will continue to shift. However, cases such as our patient may demonstrate the utility of novel antiangiogenics in the treatment of these rare neoplasms, particularly when the tumor is unresectable or necessitates subtotal resection.