**1. Introduction**

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142 Updates in Hemodialysis

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Acquired cystic disease of the kidney (ACDK) was first described in patients dying from Brights disease by John Simon in 1847 [1]. It was rediscovered and reported in 1977 in a study of 14 kidneys in 30 hemodialysis patients, and six of the 14 patients had renal cell carcinoma (RCC) [2]. Miller et al. subsequently reported a large autopsy series of 155 hemodialysis patients in which ACDK was noted in 58% and RCC in 2% of patients [3].

ACDK must be distinguished from other acquired renal cystic diseases including simple renal cysts that develop as age advances, and the cystic changes found with primary hyperaldos‐ teronism that causes hypokalemia. It must be distinguished from hereditary autosomaldominant polycystic disease, and from cystic kidney disease such as Von-Hipple-Lindau disease, tuberous sclerosis, medullary cystic disease, autosomal recessive polycystic disease, and medullary sponge kidney [4]. ACDK is defined as the presence of more than three cysts in either or both of the kidneys or the presence of cysts occupying more than 25% of the renal parenchyma in patients with end-stage renal disease (ESRD) [5, 6]. ACDK increases in prevalence and severity with increasing years on dialysis: 20% of patients dialyzed for 1 to 3 years have ACDK, compared with greater than 90% of patients dialyzed for 5 to 10 years [7]. Time spent on hemodialysis is the most important key features of developing ACDK and increasing risk for RCC [8, 9]. The most important predisposing factor for ACDK is a duration of dialysis of 5 to 10 years or longer [6, 10]. Reportedly, the incidence of ACDK in peritoneal dialysis is almost equal to that in hemodialysis patients [11, 12].

In the current practice, periodic screening for RCC is recommended for dialysis patients because of high incidence of RCC. Screening is recommended from the start of hemodialysis. Ultrasonography (US), one of standard screening tools of RCC in dialysis patients, sometimes fails to distinguish RCC from hemorrhagic cysts [13]. The usefulness of magnetic resonance

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imaging (MRI) without the use of contrast material has been reported for the detection of RCC in patients with ACDK [14]. The principle treatment strategy for RCC in dialysis patients is radical nephrectomy (RN). Long-term dialysis patients are at high risk for cardiovascular events [4]. Therefore, less invasive surgery is preferable to avoid postoperative systemic complications. Pathologically, clear and papillary RCC had been considered as common histological types of renal cancer arising from acquired cystic disease (ACD) [15, 16]. Recently, a novel standard pathologic entity of ACD-associated RCC has been established [10, 17-19].

In this chapter, we overview the upated topics on RCCs in ESRD patients, particularly focusing on screening and diagnosis, minimally invasive surgery, and pathology.
