VHL and nonpapillary renal cell carcinoma: ccRCC constitutes a typical manifestation of several familial renal cell cancer syndromes (e.g., Birt-Hogg-Dubé and constitutional chromosome 3 translocation syndromes) but occurs mainly in von Hippel-Lindau disease (VHL), a syndrome caused by germline mutations of the VHL tumor suppressor gene, located on chromosome 3p25-26 [3].