Patients with Von Hippel-Lindau (VHL) disease can develop renal cell carcinoma along with other VHL-related tumours. When surgery is not appropriate, a distinct management approach is indicated.
In VHL disease, renal tumours are typically monitored until defined thresholds are met. For patients where surgical intervention is not feasible, the management pathway shifts to a targeted pharmacological approach directed at the underlying molecular mechanism driving tumour growth in this syndrome.
A targeted HIF-2α inhibitor has been approved specifically for VHL-related renal and other tumours not requiring immediate surgery — the complete protocol, including selection criteria and management detail, is available in the structured regimen below.