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Medline ® Abstract for Reference 34

of 'Clinical features, diagnosis, and management of von Hippel-Lindau disease'

Growth kinetics in von Hippel-Lindau-associated renal cell carcinoma.
Jilg CA, Neumann HP, Gläsker S, Schäfer O, Ardelt PU, Schwardt M, Schultze-Seemann W
Urol Int. 2012;88(1):71-8. Epub 2011 Dec 09.
OBJECTIVE: To evaluate the growth kinetics of renal cell carcinoma (RCC) in von Hippel-Lindau (VHL) disease in a large trial by CT/MRI scan. VHL disease is a multisystemic disorder predisposing to renal cysts and cancer. There is a general assumption that VHL-associated RCC presents slower growth rates than sporadic RCC.
PATIENTS AND METHODS: We describe growth kinetics of 96 renal tumours in 64 VHL patients with analysed germline mutation (54/64 treated, 10/64 active surveillance) over a mean follow-up of 54.9 months. We calculated tumour volume, growth rate, multiplication of tumour volume per year and overall, as well as tumour volume doubling time.
RESULTS: The mean growth rate of 96 tumours was 4.4 mm/year (SD 3.2, median 4.1 mm/year), mean volume doubling time was 25.7 months (SD 20.2, median 22.2 months). We saw a median 1.4-fold increase in tumour volume per year. At treatment time point, VHL kidneys comprised 39% tumour and 15.7% cyst volume fraction. We saw no correlation between tumour size and growth parameters.
CONCLUSION: VHL-associated RCC show large variances in tumour growth behaviour. Compared to the literature, in our study the growth rates (mm/year) of RCC in VHL disease did not differ from those of sporadic RCC. Fast tumour growth increases the risk for metastases.
Department of Urology, Albert Ludwigs University of Freiburg, Freiburg, Germany. Cordula.Jilg @ uniklinik-freiburg.de