We observed a t(11;22)(q23-24;q11.2-12) and monosomy 3 in renal tumor cells from 
a 72-year-old man. The hypothesis of a primitive peripheral neuroectodermal 
tumor (PPNET) located in the kidney was promptly excluded: Histologically, the 
tumor was a clear cell renal cell carcinoma (RCC) and we did not observe an 
EWSR1 gene rearrangement. The constitutional origin of this alteration was 
established. We report on the second case of RCC in a patient with a 
constitutional t(11;22). The t(11;22)(q23;q11.2) is the main recurrent germline 
translocation in humans. Unbalanced translocation can be transmitted to the 
progeny and can cause Emanuel syndrome. Our observation alerts cancer 
cytogeneticists to the fortuitous discovery of the constitutional t(11;22) in 
tumor cells. This translocation appears grossly similar to the t(11;22)(q24;q12) 
of PPNET and should be evoked if present in all cells of a tumor other than 
PPNET. This is important when providing appropriate genetic counseling. 
Moreover, the potential oncogenic role of the t(11;22) and its predisposing risk 
of cancer are under debate. The family history of the patient revealed a 
disabled brother who died at an early age from colon cancer and a sister with 
breast cancer. This observation reopens the issue of a link between the 
constitutional t(11;22) and cancer, and the utility of cancer prevention workups 
for t(11;22) carriers.