Growth kinetics in von Hippel-Lindau-associated renal cell carcinoma

Urol Int. 2012;88(1):71-8. doi: 10.1159/000333348. Epub 2011 Dec 9.

Abstract

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.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Carcinoma, Renal Cell / genetics*
  • Carcinoma, Renal Cell / metabolism
  • Carcinoma, Renal Cell / pathology*
  • Carcinoma, Renal Cell / secondary
  • Carcinoma, Renal Cell / surgery
  • Cell Proliferation*
  • Female
  • Genetic Predisposition to Disease
  • Germ-Line Mutation*
  • Germany
  • Humans
  • Kidney Diseases, Cystic / genetics
  • Kidney Diseases, Cystic / pathology
  • Kidney Neoplasms / genetics*
  • Kidney Neoplasms / metabolism
  • Kidney Neoplasms / pathology*
  • Kidney Neoplasms / surgery
  • Kinetics
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Phenotype
  • Prognosis
  • Tomography, X-Ray Computed
  • Tumor Burden
  • Von Hippel-Lindau Tumor Suppressor Protein / genetics*
  • Von Hippel-Lindau Tumor Suppressor Protein / metabolism
  • Young Adult

Substances

  • Von Hippel-Lindau Tumor Suppressor Protein
  • VHL protein, human