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

RCC: renal cell carcinoma; VHL: von Hippel-Lindau.

* 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 who desire a more aggressive management strategy to potentially postpone or avoid future surgical interventions.

◊ Partial nephrectomy is preferred in most patients with resectable RCC. Radiofrequency ablation and cryotherapy are alternatives to surgery for select populations, such as patients who need to preserve kidney parenchyma (eg, solitary kidney, bilateral and/or multiple tumors, recurrent tumors), those with chronic kidney disease, older adults, or those with significant comorbidities.