Treatment of patients with von Hippel-Lindau disease and locoregional renal cell carcinoma

VHL: von Hippel-Lindau; RCC: renal cell carcinoma; mm: millimeters.
* Kidney transplantation has been safely used in patients with VHL disease and RCC, although experience is limited. Refer to UpToDate topics on kidney transplantation in adults.
¶ Patients ineligible for surgery or other nephron-sparing approaches include those with multiple prior surgeries or those with lesions in a solitary remaining kidney where further locoregional interventions would render the patient anephric.
Δ Belzutifan is an alternative to surveillance for patients with rapidly growing tumors or those who desire a more aggressive management strategy, in order to potentially postpone or avoid future surgical interventions.
◊ Surgery is preferred in most patients with resectable RCC. Nonsurgical options such as radiofrequency ablation and cryotherapy represent less invasive alternatives for select populations, such as older adults, those with significant comorbidities, or those with the need to preserve kidney parenchyma (eg, solitary kidney, multiple lesions). Refer to UpToDate content on radiofrequency ablation and cryoablation for RCC.