Umberto Capitanio1,2, Giuseppe Rosiello3,4, Selcuk Erdem5, Isaline Rowe4, Onder Kara6, Eduard Roussel7, Riccardo Campi8,9, Tobias Klatte10, Maximilian C Kriegmair11, Mattia Sibona12, Riccardo Bertolo13, Idir Ouzaid14, Maria Carmen Mir15, Giancarlo Marra12, Alessandro Larcher3,4, Francesco Montorsi3,4, Andrea Salonia3,4. 1. Unit of Urology, University Vita-Salute, IRCCS San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy. capitanio.umberto@hsr.it. 2. Division of Experimental Oncology, URI, Urological Research Institute, IRCCS San Raffaele Scientific Institute, Milan, Italy. capitanio.umberto@hsr.it. 3. Unit of Urology, University Vita-Salute, IRCCS San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy. 4. Division of Experimental Oncology, URI, Urological Research Institute, IRCCS San Raffaele Scientific Institute, Milan, Italy. 5. Department of Urology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey. 6. Urology Department, Kocaeli University School of Medicine, Kocaeli, Turkey. 7. Department of Urology, University Hospitals Leuven, Leuven, Belgium. 8. Department of Urology, University of Florence, Careggi Hospital, Florence, Italy. 9. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. 10. Department of Urology, Department of Surgery, Royal Bournemouth Hospital, University of Cambridge, Cambridge, UK. 11. Department of Urology, University Hospital Mannheim, University of Heidelberg, Heidelberg, Germany. 12. Department of Urology, San Giovanni Battista Hospital, University of Turin, Turin, Italy. 13. Department of Urology, San Carlo Di Nancy Hospital, Rome, Italy. 14. Department of Urology, Bichat University Hospital, Paris-Didert University, Paris, France. 15. Department of Urology, Fundacion Instituto Valenciano Oncologia, Valencia, Spain.
Abstract
PURPOSE: To investigate the natural history and follow-up after kidney tumor treatment of Von Hippel-Lindau (VHL) patients. MATERIALS AND METHODS: A multi-institutional European consortium of patients with VHL syndrome included 96 non-metastatic patients treated at 9 urological departments (1987-2018). Descriptive and survival analyses were performed. RESULTS AND LIMITATIONS: Median age at VHL diagnosis was 34 years (IQR 25-43). Two patients (2.1%) showed only renal manifestations at VHL diagnosis. Concomitant involvement of Central Nervous System (CNS) vs. pancreas vs. eyes vs. adrenal gland vs. others were present in 60.4 vs. 68.7 vs. 30.2 vs. 15.6 vs. 15.6% of patients, respectively. 45% of patients had both CNS and pancreatic diseases alongside kidney. The median interval between VHL diagnosis and renal cancer treatment resulted 79 months (IQR 0-132), and median index tumor size leading to treatment was 35.5 mm (IQR 28-60). Of resected malignant tumours, 73% were low grade. Of high-grade tumors, 61.1% were large > 4 cm. With a median follow-up of 8 years, clinical renal progression rate was 11.7% and 29.3% at 5 and 10 years, respectively. Overall mortality was 4% and 7.5% at 5 and 10 years, respectively. During the follow-up, 50% of patients did not receive a second active renal treatment. Finally, 25.3% of patients had CKD at last follow-up. CONCLUSIONS: Mean period between VHL diagnosis and renal cancer detection is roughly three years, with significant variability. Although, most renal tumors are small low-grade, clinical progression and mortality are not negligible. Moreover, kidney function represents a key issue in VHL patients.
PURPOSE: To investigate the natural history and follow-up after kidney tumor treatment of Von Hippel-Lindau (VHL) patients. MATERIALS AND METHODS: A multi-institutional European consortium of patients with VHL syndrome included 96 non-metastatic patients treated at 9 urological departments (1987-2018). Descriptive and survival analyses were performed. RESULTS AND LIMITATIONS: Median age at VHL diagnosis was 34 years (IQR 25-43). Two patients (2.1%) showed only renal manifestations at VHL diagnosis. Concomitant involvement of Central Nervous System (CNS) vs. pancreas vs. eyes vs. adrenal gland vs. others were present in 60.4 vs. 68.7 vs. 30.2 vs. 15.6 vs. 15.6% of patients, respectively. 45% of patients had both CNS and pancreatic diseases alongside kidney. The median interval between VHL diagnosis and renal cancer treatment resulted 79 months (IQR 0-132), and median index tumor size leading to treatment was 35.5 mm (IQR 28-60). Of resected malignant tumours, 73% were low grade. Of high-grade tumors, 61.1% were large > 4 cm. With a median follow-up of 8 years, clinical renal progression rate was 11.7% and 29.3% at 5 and 10 years, respectively. Overall mortality was 4% and 7.5% at 5 and 10 years, respectively. During the follow-up, 50% of patients did not receive a second active renal treatment. Finally, 25.3% of patients had CKD at last follow-up. CONCLUSIONS: Mean period between VHL diagnosis and renal cancer detection is roughly three years, with significant variability. Although, most renal tumors are small low-grade, clinical progression and mortality are not negligible. Moreover, kidney function represents a key issue in VHL patients.