In UTUC etiology, genetic susceptibility and gene-environmental interaction or differences in the ability to counteract carcinogens has been suggested [1], however, little is known about the susceptibility loci to UTUC and so far only two UTUC-specific SNPs have been reported, including the variant allele SULT1A1*2 reducing sulfotransferase activity and the T-allele of rs9642880 on chromosome 8q24 [1, 8]. Here, SULT1A1 is linked to renal pelvis/ureter urothelial carcinoma.