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

Multiple recurrent renal cell carcinomas driven by germline VHL variants, requiring a repeated nephron-sparing surgical strategy.

von Hippel-Lindau disease predisposes to recurrent renal cell carcinoma requiring repeated renal interventions, whose timing and long-term outcomes were poorly documented. This retrospective single-centre analysis covers 89 patients undergoing repeated renal interventions for renal cell carcinoma, including nephron-sparing surgery, nephrectomy and minimally invasive procedures, with kidney- and patient-based Kaplan-Meier analyses. A total of 232 interventions were performed, a median of two procedures per patient, with median follow-up of 14.6 years; among the 204 surgical interventions (167 nephron-sparing procedures, 37 nephrectomies), operative time, blood loss and length of stay were comparable. Kidney-based analysis (n = 178) showed median intervals of 10.7 and 8.2 years between successive ipsilateral procedures, with nephrectomy occurring after a median of 24.6 years, while patient-based analysis gave median times of 4.9, 6.4 and 2.6 years to the second, third and fourth interventions. Metastases occurred in 10 patients, median overall survival from first intervention was 20.9 years, and complications, more frequent after nephron-sparing surgery, were rarely Clavien-Dindo grade 4 or higher.

Synthesis written by Geno'X. For the full original abstract, please refer to the source publication.

This is the series that was missing to answer the question VHL carriers actually ask in clinic, namely the expected pace of repeat surgery: median intervals of 4.9 then 6.4 years, with nephrectomy deferred beyond twenty years, are figures that can be used directly when informing patients. The trade-off is recruitment from a single expert centre in selected patients, reflecting the best-case scenario rather than average practice, hence the authors' referral recommendation. The series settles neither the choice between surgery and ablative techniques nor the role of systemic therapies targeting the VHL pathway in deferring intervention.

Analysis by Dr Thibaut Benquey