Repeated Renal Interventions for Renal Cell Carcinoma in von Hippel-Lindau Disease: Long-Term Outcomes

Abstract

Von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome frequently associated with recurrent renal cell carcinoma (RCC), often requiring multiple renal interventions. Data on the timing and outcomes of repeated renal procedures remain limited. This study aimed to characterize patterns, timing, and outcomes of repeated renal interventions in VHL disease. Retrospective single-center analysis of 89 patients with VHL undergoing repeated renal interventions for RCC, including nephron-sparing surgery (NSS), nephrectomy, and minimally invasive interventions. Time to repeated interventions was assessed using kidney- and patient-based Kaplan-Meier analyses. Outcomes included perioperative parameters, metastasis, and overall survival. A total of 232 renal interventions were performed, with a median of two procedures per patient and a median follow-up of 14.6 yr. Among 204 surgical interventions (167 NSS, 37 nephrectomies), operative time, blood loss, and length of hospital stay were comparable. Kidney-based analyses (n = 178) showed median intervals of 10.7 and 8.2 yr between successive ipsilateral procedures, while nephrectomy occurred after a median of 24.6 yr. Patient-based analyses demonstrated median times of 4.9, 6.4, and 2.6 yr to the second, third, and fourth interventions. Metastases occurred in 10 patients, and median overall survival from first intervention was 20.9 yr. Perioperative complications were more frequent after NSS, whereas high-grade complications (Clavien-Dindo grade 4 or higher) were rare. Limitations include the single-center design and selected patient population. Repeated renal interventions are common in VHL disease. Organ-preserving strategies allow long-term renal preservation with acceptable oncologic outcomes and low rates of severe complications. Referral to experienced centers is recommended.