Renal cell carcinoma (RCC) is a common manifestation in Von-Hippel Lindau (VHL) syndrome, accounting for almost 30% of the patients. However, since VHL syndrome is relatively rare, there are no clear guidelines regarding the approach to VHL patients with RCC. This article aims to provide evidence-based insights for diagnosis, treatment, and surveillance in VHL patients with RCC.
The articles indexed between 1977 and 2025 in PubMed was searched. Only articles in English were included.
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. Due to recurrent characteristics of RCC, nephron-sparing surgeries should be prioritized with the aim of life-long kidney function preservation, whenever it is feasible. Focal treatments should be considered in patients for whom surgery is not suitable. In addition, belzutifan treatment has shown promising results in selected patients.
In VHL patients with RCC, kidney function should be preserved whenever possible to ensure a long and high-quality life. Since these patients may develop lesions in other organs beyond kidney, surveillance with multidisciplinary approach in tertiary centers should be recommended.
World journal of urology. 2026 Jun 29*** epublish ***
Selcuk Erdem, Daniele Amparore, Chiara Re, Samed Verep, Umberto Capitanio, Alessandro Larcher, Eduard Roussel, Tobias Klatte, Riccardo Bertolo, Stijn Muselaers, Nicola Pavan, Onder Kara, Alexandre Ingels, Angela Pecoraro, Michele Marchioni, Savio Domenico Pandolfo, Pietro Diana, Umberto Carbonara, Veronica Mollica, Hannah Warren, Leonardo Borregales, Laura Marandino, Gopal Sharma, Anna Caliò, Zhenjie Wu, Luca Alberto Lovati, Chiara Ciccarese, Gaëlle Margue, Giulio Francolini, Ronan Flippot, David Kawai Leung, Georges Mjaess, Giuseppe Rosiello, Maria Carme Mir, Carlotta Palumbo, Riccardo Campi, EAU-YAU Renal Cancer Working Group
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