Abstract
Renal cell carcinoma (RCC) occurring in the setting of end-stage renal disease (ESRD) shows unique clinicopathological characteristics. The two most frequent types of ESRD-associated RCC are acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC) and clear-cell papillary renal cell carcinoma (ccpRCC). Other types of RCC also occur in ESRD, albeit with different frequencies from the non-ESRD general population. The histological features of RCC do not vary in the setting of ESRD vs. non-ESRD, yet other findings, such as multifocality and multiple tumor types, are more frequent in ESRD. Studies have generated novel and important knowledge of the etiology, epidemiology, diagnosis, treatment, immunophenotype, and molecular characteristics of ESRD-associated RCC. Knowledge of these data is important for both pathologists and other physicians who may encounter ESRD patients with RCC. This review presents a comprehensive summary and update of the literature on RCC in ESRD, with a focus on the two most frequent types, ACKD-RCC and ccpRCC.
Highlights
The prevalence of end-stage renal disease (ESRD) requiring renal replacement therapy is estimated at 4–7 million cases worldwide [1] and the prevalence of ESRD continues to rise [2]
Acquired cystic kidney disease (ACKD) is a condition which develops in ESRD patients characterized by cystic dilation of renal tubules and is associated with a unique type of RCC, namely, acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC) [6]
The majority of RCCs occurring in ESRD and/or ACKD represent ACKD-RCC in addition to another unique RCC entity, clear-cell papillary renal cell carcinoma
Summary
The prevalence of end-stage renal disease (ESRD) requiring renal replacement therapy is estimated at 4–7 million cases worldwide [1] and the prevalence of ESRD continues to rise [2]. It has been recognized that patients with ESRD have an increased propensity for developing renal cell carcinoma (RCC) at rates higher than patients without ESRD [3–5]. Acquired cystic kidney disease (ACKD) is a condition which develops in ESRD patients characterized by cystic dilation of renal tubules and is associated with a unique type of RCC, namely, acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC) [6]. ACKD-RCC and ccpRCC are recognized entities in the 2016 World Health Organization classification of renal tumors [7]. This review details knowledge of the clinicopathological characteristics of renal tumors associated with ESRD, with special emphasis on ACKD-RCC and ccpRCC. It provides an update on our evolving knowledge, which will be useful for pathologists and physicians who may encounter and treat patients with these tumors. Percentages for each tumor type are calculated as number of tumors per total number of ESRD kidneys. (As some tumors occurred multiply in the same kidney, the sum of percentages calculated is not 100%)
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