What do we know about the Balkan endemic nephropathy and the uroepithelial tumors?

Balkan endemic nephropathy (BEN), a familial chronic tubulo interstitial disease with a slow progression to terminal renal failure, affects people living in the alluvial plains along the tributaries of the Danube River. One of its most peculiar characteristics is a strong association with upper urothelial cancer. An increased incidence of upper urinary tract (UUT) transitional cell cancer (TCC) was discovered among the inhabitants of endemic settlements and in families affected by BEN. In areas where BEN is endemic, the incidence of upper tract TCC is significantly higher, even 100 times, than in non-endemic regions. Until now, several hypotheses have been introduced about the etiopathogenesis of BEN. Only the toxic effect aristolochia clematidis has been confirmed as a factor in the occurrence of the disease. We don't have specific biomarkers for an early diagnosis of BEN and UUT-TCC. With application of modern molecular and genetic methods in investigation of etiopathogenesis and diagnosis of BEN and UUT-TCC we should expect improvement in the study of BEN.