Introduction: End-stage renal disease (ESRD) and acquired cystic kidney disease (ACKD) are known risk factors for renal cell carcinoma (RCC). Hereby, the clinicopathological features of RCCs developed in ESRD were investigated. Methods: A database consisting of 34 tumors from 31 patients with ESRD among 2 566 nephrectomy samples of RCC was built. The demographic, clinical, and follow-up data along with pathological parameters were analyzed. The RCCs were diagnosed according to the current WHO Classification of Urinary and Male Genital Tumors. Results: Twenty-two tumors developed in men and 12 in women, with a median age of 56 years (range: 27-75 years). The causes of ESRD were glomerulonephritis (n=7), hypertensive kidney disease (n=6), autosomal dominant polycystic kidney disease (n=6), chronic pyelonephritis (n=4), diabetic nephropathy (n=3), chemotherapy-induced nephropathy (n=1), and undetermined (n=4). ACKD complicated ESRD in 12 patients. The following histological subtypes were identified: clear cell RCC (n=19), papillary RCC (n=5), clear cell papillary tumor (n=5), ACKD RCC (n=3), and eosinophilic solid and cystic RCC (n=2). The median tumor size was 31 mm (range: 10-80 mm), and 32 tumors were confined to the kidney (pT1-pT2). There was no tumor-specific death during the period of this study. Progression was registered in one patient. Conclusion: In our cohort, the most common RCC subtype was clear cell RCC (55%), with a frequency that exceeded international data appreciably (14-25%). The incidence of clear cell papillary tumor and ACKD RCC (14.7% and 8.5%) was lower than data reported in the literature (30% and 40%). Our results indicate a favorable prognosis of RCC in ESRD.

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