Upper urinary tract tumours are exceptional in the context of renal polycystic disease. The authors report the case of Mrs B. F., 56 years old, who presented with left loin pain associated with haematuria. Clinical examination was normal and ultrasound examination revealed bilateral renal polycystic disease with a mass in the left renal sinus. CT urography showed a tumour arising from the renal pelvis suggestive of an upper urinary tract tumour. The laboratory assessment revealed normal renal function and normal urine cytology. Treatment consisted of radical nephroureterectomy with resection of a bladder cuff. Histological examination revealed a urothelial tumour of the renal pelvis with negative surgical margins. In the light of this case, the authors discuss the diagnostic difficulties and specificities, the treatment and the outcome of this unusual clinical association.
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