In approximately 40% of sporadic clear cell RCC, the inactivation of the von Hippel–Lindau (VHL) tumour suppressor gene located on chromosome 3p25 has been observed and seems to play a major role for the development of this tumour type (Foster et al, 1994, Gnarra et al, 1994, Brauch et al, 2000, Mandriota et al, 2002). The gene discussed is VHL; the disease is neoplasm.