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New research · Nephrology
World journal of urology · 2d
GuidelineWorld journal of urology · 2026

What should the urologist know on the management of kidney cancer in patients with Von Hippel-Lindau syndrome? Recommendations of the European Association of Urology (EAU) Young Academic Urologists (YAU) Renal Cancer Working Group.

Selcuk Erdem, Daniele Amparore, Chiara Re … EAU-YAU Renal Cancer Working Group
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NephrologyGuideline

Renal cell carcinoma affects almost 30% of patients with Von Hippel-Lindau syndrome.

What should the urologist know on the management of kidney cancer in patients with Von Hippel-Lindau syndrome? Recommendations of the European Association of Urology (EAU) Young Academic Urologists (YAU) Renal Cancer Working Group.

Selcuk Erdem et al. · World journal of urology · 2026
Background

Renal cell carcinoma (RCC) is a common manifestation in Von-Hippel Lindau (VHL) syndrome, accounting for almost 30% of the patients.

Purpose

This article aims to provide evidence-based insights for diagnosis, treatment, and surveillance in VHL patients with RCC.

Methods

The articles indexed between 1977 and 2025 in PubMed was searched.

Results
people with VHL syndrome are affected by kidney cancer
More results

Renal cysts are quite common in VHL patients and these cysts predispose for RCC.

VHL patients are characterized with early-onset, multifocal and/or bilateral, recurrent and clear cell subtype RCC.

Patients should be monitored at regular intervals.

More results

Focal treatments should be considered in patients for whom surgery is not suitable.

“
Conclusion · 1 of 2

In VHL patients with RCC, kidney function should be preserved whenever possible to ensure a long and high-quality life.

Conclusion · 2 of 2

Since these patients may develop lesions in other organs beyond kidney, surveillance with multidisciplinary approach in tertiary centers should be recommended.

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