Acquired cystic disease of the kidney and renal cell carcinoma

This book is a well-presented and data-rich compendium on theproblem of acquired cysts and kidney cancers that occur in failedend-stage kidneys. Its Japanese author, Dr Isao Ishikawa, isthe world's authority on this subject. This book shows his comprehensive     

Acquired cystic disease of kidney in chronic dialysis patients

Eight cases of acquired cystic disease of the kidney (ACDK) associated with chronic renal failure and hemodialysis are described. No patient had a family history or clinical evidence of congenital adult polycystic kidney disease (CAPKD). Glomerulonephritis was the cause of renal failure in 6, and pyelonephritis in 2. Massive renal and perirenal hemorrhage necessitated 3 nephrectomies in 2 patients. Single kidney weights did not exceed 280 Gm., a major feature in the distinction of ACDK from CAPKD. Morphologically, in addition to the usual stigmata of end-stage kidneys, 40 to 80 per cent of the renal parenchyma was replaced by small cysts. Continuity of cysts with tubules was established by nephron dissection.     

Acquired cystic kidney disease in children undergoing long-term dialysis

Acquired cystic kidney disease (ACKD) occurs in adult patients undergoing long-term dialysis. Early detection is important because clinically significant hematuria and malignancies are associated with ACKD. We evaluated by magnetic resonance imaging (MRI) and ultrasonography (US) the incidence of ACKD in 15 patients aged 7.3–21.6 years (mean 15.9 years) with non-cystic primary renal disease. Nine patients had been treated with peritoneal dialysis only, and 6 with both hemodialysis and peritoneal dialysis for 24–73 months (mean 37 months). Three patients (20%) had no cysts. In 5 patients (33%) with bilateral multiple cysts, the diagnosis of ACKD was made by MRI and US. In another 5 patients, solitary cysts were localized to one kidney by MRI, and in 2 patients solitary cysts were seen in both kidneys. This study documents that ACKD is not limited to older patients with end-stage renal disease. Early detection of these cysts can be accomplished by MRI and is warranted since 1 patient developed neoplastic tubular changes which can precede tumor formation.     

Acquired renal cystic disease in the end stage kidney: urological implications

A unique form of acquired renal cystic disease occurs commonly in the end stage kidneys of patients with chronic renal failure. Recent experience with 3 cases of acquired renal cystic disease has made us aware that the condition has significant urological implications. The pathogenesis of this disease is unknown but may be related to tubular obstruction, ischemia or the accumulation of toxic products. The diagnosis of acquired renal cystic disease is established by either ultrasound or computerized tomography, both of which demonstrate bilateral multiple small cysts scattered throughout the cortex and medulla of the contracted end stage kidney. Acquired renal cystic disease usually is asymptomatic but may be associated with either hemorrhage or neoplasia. Autopsy studies have revealed renal tumors in up to 45 per cent of the patients with acquired renal cystic disease. These tumors usually are small but our case 3 was a renal cell carcinoma that measured 4 cm. in diameter. Also, there have been other recent reports of large tumors and deaths of metastatic renal carcinoma in patients with acquired renal cystic disease. Patients with chronic renal failure should undergo periodic examination of the native kidneys by either ultrasound or computerized tomography. It may be difficult to distinguish benign from malignant lesions radiologically, and nephrectomy may be indicated when the diagnosis is uncertain.     

Acquired renal cystic disease

In 1977, Dunnill et al. described a new disorder, bilateral multiple renal cystic disease. It occurred among hemodialysis patients whose original illness had not been cyst-related. Acquired cystic disease of the kidney (ACDK) is commonly observed in patients undergoing hemodialysis. The incidence of ACDK is 40-50% in reports of autopsy and surgical specimens, rising to more than 90% after 5-10 years of dialysis. The volume of the kidneys decreases in the first 3 years of dialysis and then increases as the rate of cyst formation increases. In male patients undergoing long-term hemodialysis the incidence of ACDK is markedly high. ACDK is also found in patients before hemodialysis. The primary concern in patients with ACDK is the increased incidence (5-19%) of renal cell carcinoma (RCC). The incidence is about twelve to eighteen times higher than that in the general population and the cancers may be asymptomatic. Therefore, screening is essential if carcinomas are to be detected early. Regular screening by ultrasonic examination or CT scan is needed. A patient requires nephrectomy when the kidney cancer exists or is suspected by dynamic CT scan. Nephrectomy is performed only on the side with renal mass. It has been argued that RCC associated with ACDK are innocuous and do not predispose the patient to an increased risk of death from RCC. RCC arising from ACDK is considered to be a tumor of low malignant potential, compared with classic RCC. However, RCC has been reported to metastasize in 16% of the patients on dialysis and to be the cause of death in 2% of the kidney transplant recipients. The etiology of ACDK is unclear and its incidence increases with the duration of dialysis. ACDK patients have a propensity to develop adenocarcinoma. The increased incidence of RCC in ACDK patients warrants careful radiologic monitoring of end-stage kidneys in selected patients.     

Acquired cystic disease of the kidney in association with chronic ambulatory peritoneal dialysis

A combined necropsy and ultrasound study in patients with end-stage renal disease treated exclusively by peritoneal dialysis revealed acquired cystic disease of the kidney (ACDK) in five of 15 necropsies and in one of seven sonograms from living patients. Two benign microscopic adenomas were also found in the first group of patients. No malignant renal tumors or hemorrhagic complications were detected. The cause of the cyst formation is clearly related to chronic renal failure rather than dialysis per se, as one patient had cysts prior to CAPD and seven of 41 patients with end-stage renal disease in the predialysis era were found to have renal cysts on postmortem examination. This study shows that ACDK is not uncommon in patients with chronic renal failure treated by chronic ambulatory peritoneal dialysis.     

Acquired cystic kidney disease: rapid progression from small to enlarged kidneys simulating adult polycystic kidney disease

A 57-year-old man on chronic hemodialysis presented marked bilateral renal enlargement due to acquired cystic kidney disease (ACKD). He had been on hemodialysis for less than 3 years only (14 months prior to receiving a functional renal transplant which lasted 8 years, followed by 18 additional months of dialysis), before the diagnosis of ACKD was made following an episode of flank pain with gross hematuria. The marked changes in kidney appearance during this 11-year period were documented by serial ultrasound examination showing the kidneys to be of near-normal size before the start of dialysis (> or =10 cm in 1986), then shrunken and contracted 5 years later while having a functioning renal transplant (<5 cm in 1991), and markedly enlarged reaching the size of adult polycystic kidney disease after returning to dialysis (>13 cm in 1997). Since the risk of ACKD increases with duration of dialysis, we sought additional predisposing factors in this unusual case and found that 2 years after renal transplantation, the patient was diagnosed with breast cancer for which he was treated with surgical excision and tamoxifen. Based on ultrasound evidence that the tamoxifen treatment preceeded the appearance of the renal cystic changes, we wonder whether this drug may have played a role in the rapid development of ACKD.     

Acquired cystic kidney disease following long-term peritoneal dialysis for congenital nephrotic syndrome

We describe here the clinicopathological findings in a child with congenital nephrotic syndrome (CNS) non-responsive to medical therapy who developed acquired cystic kidney disease (ACKD) in both native kidneys after long-term peritoneal dialysis. This case indicates that CNS is a further pathologic condition related to the development of ACKD.     

Acquired cystic kidney disease: occurrence in patients on chronic peritoneal dialysis

Acquired cystic kidney disease (ACKD) is a well-known complication of long-term hemodialysis. To the best of our knowledge, only six patients on continuous ambulatory peritoneal dialysis have been reported to develop this disease. We herein report two such cases, and concluded that the morphology of ACKD seems to be independent of the type of dialysis and that hemodialysis is not necessary for the development of ACKD.     

Acquired renal cystic disease mimicking adult polycystic kidney disease in a patient undergoing long-term hemodialysis.

Acquired renal cystic disease (ARCD) is defined as the development of multiple cysts in the renal cortex and medulla in patients with chronic renal failure who are free from congenital polycystic kidney disease. ARCD develops generally in contracted kidneys. We report a case of grotesque enlargement of a single kidney in a patient who had been receiving hemodialysis for 18 years. Although the exact causes of ARCD are not known, 3 factors may contribute to the development of nephromegaly; the sex, the duration of hemodialysis and previous unilateral nephrectomy. As in polycystic disease, when the involved kidney reaches considerable size, ARCD may have a favorable effect on anemia caused by chronic renal failure.     

Acquired cystic disease of the kidney and renal carcinoma in haemodialysis patients: ultrasonographic evaluation.

Ultrasonography was performed in 661 dialysis patients and acquired cystic disease of the kidney was found in 156 (125 men and 31 women). A higher incidence of cystic disease was found in males. There was no significant difference between the patients with and those without acquired cystic disease in terms of average age, but the duration of haemodialysis in those with acquired cystic disease was significantly longer. There was an increased incidence of cystic disease in patients with glomerulonephritis and the duration of haemodialysis in these patients was significantly longer. This suggests that the increased incidence of acquired cystic disease of the kidneys in the patients with glomerulonephritis is simply related to the longer duration of treatment. Twelve patients with renal carcinoma were found in this study. The average age at diagnosis of renal carcinoma was not significantly different between the patients with and those without acquired cystic disease, but the duration of dialysis was significantly longer in renal carcinoma patients with acquired cystic disease. The incidence of renal carcinoma in dialysis patients with acquired cystic disease was 3.85% and in those without it was 1.19%. These rates are considerably higher than those found in the general population and indicate that the risk of renal carcinoma is higher in dialysis patients both with and without acquired cystic disease.     

Acquired cystic disease of kidney associated with renal cell carcinoma in chronic dialysis patients.

We describe 2 cases of acquired cystic disease of the kidney (ACDK) associated with renal cell carcinoma in patients treated with long-term hemodialysis. Both patients have had dialysis for five and eight years, respectively. Renal cell carcinomas of these patients are small, averaging 2 cm in diameter. They are clear cell type. Atypical epithelial hyperplasia arising from cystic areas can be seen intermingling with carcinoma. This confirms that atypical epithelial hyperplasia is a precursor of renal cell carcinoma. Although the incidence of renal cell carcinoma arising in ACDK is on the rise, the issue of how to manage patients with ACDK remained unsettled and required further study.     

Acquired renal cystic disease: implications for the urologist

Acquired cystic kidney disease has become increasingly recognised as a significant risk in patients with end-stage renal disease, especially in those maintained on chronic haemodialysis and peritoneal dialysis. A review of the literature indicates that nearly 50% of patients on dialysis for more than 3 years develop renal cystic changes. The major complications of this condition are neoplasia and spontaneous renal haemorrhage. The risk of developing renal carcinoma has been estimated to be more than 30 times higher in dialysis patients with cystic changes than in the general population. Our experience with 5 patients is reported, including 3 with renal tumours and 1 with metastatic disease. Careful surveillance of dialysis patients using yearly ultrasonography and computed tomography is recommended. The evolving indications for radical nephrectomy in this disease are discussed.     

Uremic acquired cystic disease of kidney

Multiple cystic disease occurring in the diseased kidneys of patients with end-stage renal insufficiency is called uremic acquired cystic disease of the kidney. In male patients undergoing long-term hemodialysis the incidence of ACDK is markedly high. ACDK is known to be accompanied by tumor, bleeding, calculus, abscess, etc., and the complication of cancer of the kidney is a special problem. In patients undergoing hemodialysis, occurrence of ACDK, tumor, and kidney cancer are observed respectively at the rate of 47.1, 4.8, and 1.5 per cent. When hemodialysis patients show gross hematuria, flank pain, rapid decrease in hematocrit, and sustained fever, ACDK or its complications should be investigated. Since the risk accompanied by kidney cancer is high in spite of a lack of symptoms, regular screening by ultrasonic examination or CT scan is needed. Renal transplantation is also recommended because of the regression of ACDK after successful renal transplantation. In the future, it appears that ACDK should be considered one disease entity and added to the categories of renal cystic diseases. In addition, ACDK can be studied as a model for clarification of the mechanism of cyst and tumor occurrence.     

Acquired glomerulocystic kidney disease following haemolytic-uraemic syndrome

Glomerulocystic kidney disease is a rare condition, usually seen in infants and young children, characterised by cystic dilatation of the glomeruli. It may be sporadic or represent the congenital expression of dominant polycystic kidney disease. Glomerular cysts may also be seen in association, with various syndromes and as a component of dysplastic kidneys. Only two cases of acquired glomerulocystic kidneys have been described, both in adults. The cystic change followed haemolytic-uraemic syndrome in one patient and systemic sclerosis in the other. These two conditions are closely related and may be indistinguishable pathologically. We report a case of acquired glomerulocystic kidney in a child which followed the haemolytic-uraemic syndrome. The factors leading to glomerulocystic kidney following haemolytic-uraemic syndrome are unknown and need further evaluation.