A patient with von Hippel-Lindau (VHL) disease presents with bilateral multifocal clear cell renal cell carcinomas, all <3 cm. The preferred management strategy to preserve maximal renal function is:

• A Bilateral radical nephrectomy with immediate renal transplant listing
• B mTOR inhibitor therapy alone to control tumor growth
• C Active surveillance until any lesion reaches 3 cm, then nephron-sparing surgery ✓
• D Percutaneous radiofrequency ablation of all lesions immediately

Explanation

VHL-associated RCC is typically multifocal and bilateral; repeated nephron-sparing surgery (partial nephrectomy) guided by a 3 cm threshold avoids premature dialysis while reducing metastatic risk, as lesions <3 cm rarely metastasize. This 'watch and wait until 3 cm, then operate' protocol was established in the NCI's VHL cohort and preserves renal parenchyma for the multiple surgeries these patients require over their lifetime. Radical nephrectomy prematurely commits patients to renal replacement therapy.

Reference: Bailey & Love's Short Practice of Surgery, 27th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.