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 renal cystic disease after liver transplantation in children

To our knowledge, the development of renal cystic disease that may contribute to kidney dysfunction has never been reported after liver transplantation. Herein we have reported on the fortuitous finding of renal cystic lesions upon computed tomographic scans (CT) in 33 (30%) of 108 pediatric liver transplant recipients who were the subjects of a prospective study evaluating long-term kidney dysfunction at 10 years after liver transplantation. The renal lesions had 2 different appearances: that of simple renal cysts and that of round lesions that were spontaneously hyperdense before contrast injection. These high-density lesions had a low signal on T2 weighted sequences, but 70% of them had been missed at ultrasonography. Their aspect upon CT and magnetic resonance favored cystic lesions filled with hemorrhagic or milk calcium content. Both types of cystic lesions were associated in 14 children. The renal lesions were significantly associated with moderate renal dysfunction, biopsy-proven chronic liver graft rejection, and thrombosis of the retrohepatic vena cava. The physiopathology of these lesions is undetermined. Two important questions need to be clarified with respect to the risk of progression of renal dysfunction associated with individual volume changes and/or increased number of renal cysts, as well as the risk of renal cancer as has been reported in dialyzed patients with acquired cystic kidney disease.     

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.     

Acquired renal cystic disease in end-stage renal disease: an autopsy study of 155 cases

Autopsy reports on 155 successive end-stage renal disease (ESRD) patients and 147 control patients without ESRD, matched for age, race and gender, were collected. Cystic transformation of the kidneys was noted in 58% of the ESRD patients and 13.6% of the control patients. There were 25 ESRD patients with renal adenoma and 3 with renal cell carcinoma. The single best predictor of cystic transformation or tumorous degeneration was patient's age. A statistically significant association between cystic transformation and gender, as well as the cause of ESRD, was also found. In contrast to previous reports we were unable to document a strong association between the incidence of either cystic transformation or tumorous degeneration with the duration of dialysis, nor did these two parameters correlate with mode of dialysis. We suggest that cystic transformation of the kidneys in ESRD is related to age and renal failure per se, is not strongly associated with duration of dialysis and is independent of mode of dialysis. Concomitant tumorous degeneration was frequent, but this was usually an incidental autopsy finding. Renal malignancy was uncommon and metastases were absent.     

获得性肾囊性疾病合并肾癌11例临床分析

目的 探讨获得性肾囊性疾病(ACKD)合并肾癌的诊断治疗策略.方法 回顾性分析11例终末期肾衰竭、获得性肾囊肿合并肾癌患者的临床资料.男8例,女3例.平均年龄55(37～68)岁.行血液透析至发现肾脏病变时间平均为4.8(2.8～7.4)年.结果 11例均行肾癌根治术.术后病理报告:透明细胞癌3例,乳头状癌6例,嫌色细胞癌1例,乳头状腺瘤1例.病理分期T1a9例,T1b 2例.11例术后随访平均55(17～83)个月.1例术后24个月发现肺转移;1例单侧发病者术后22个月对侧复发,行肾癌根治术;1例死于心血管疾病;1例随访19个月后失访;无瘤生存7例.结论 ACKD与肾癌有较高的相关性.终末期肾衰竭患者透析前氮质血症时间较长或透析时间＞3年者,应排除ACKD.超声及CT检查对早期诊断存在价值.除关注ACKD恶性变倾向外,对长期肾衰竭患者的其他并发症如心脑血管疾病、糖尿病等也应足够重视并行积极治疗.

Abstract：

Objective To discuss the diagnosis and treatment of acquired cystic kidney disease complicated by kidney cancer. Methods Clinical data of 11 patients with acquired cystic kidney disease complicated by kidney cancer were analyzed retrospectively. Eight patients were male and three were female. The mean age was 55 years old (range 37 to 68). The time of hemodialysis ranged from 2.8 to 7. 4 years, mean 4. 8 years. Results Follow-up ranged from 17- 83 months, mean 55 months. One patient died of cardiovascular disease. Lung metastasis was detected in one patient two years after surgery. Seven patients survived free of tumor recurrence and there was no follow-up on one patient. Conclusions Increased incidence of cancer was observed in patients with end-stage renal disease who have undergone long-term dialysis. In particular, renal cell carcinoma (RCC) showed an excess incidence in ACKD patients. RCC showed an increased prevalence compared with the general population. Patients with predialysis azotemia or a dialysis duration of longer than 3 years should be screened for ACKD. Sonegraphy or CT scanning are useful for early diagnosis of ACKD. We should pay close attention to complications, including ACKD malignant tendency, in patients who have been taking long-term dialysis and positive therapy.     

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 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 renal cystic disease in children and young adults on maintenance dialysis

Acquired renal cystic disease (ARCD) is a well-known complication of end-stage renal disease (ESRD). We studied 24 patients, aged 8 – 27 years (mean 19.8±5.3 years), on chronic maintenance dialysis in our service. The duration of dialysis ranged between 13 and 192 months (mean 77.8±44.3 months). High-resolution ultrasonography revealed ARCD in 11 (45.8%) patients. No cysts were seen in 7 (29.1%) patients and solitary cysts in one or both kidneys were seen in 6 (25%) patients. Renal malignancy was diagnosed in 2 patients. One, 15 years old, had renal cell carcinoma after being on dialysis for 6 years. She did well after bilateral nephrectomy, left salpingo-oophorectomy, and regional lymphadenectomy. The second patient, 23 years old, had been on dialysis for 16 years when she developed renal oncocytoma. She died of congestive cardiomyopathy 6 months later. We conclude that ARCD is common in children and young adults with ESRD. Neoplastic transformation, although rare, is a potential complication. Annual follow-up with ultrasonography with selective use of computed tomography or magnetic resonance imaging is advised. Received July 29, 1996; received in revised form and accepted November 15, 1996     

Acquired renal cystic disease in children prior to the start of dialysis

This report describes the clinical course and serial sonographic findings in three children who developed acquired renal cystic disease (ARCD) prior to the institution of dialysis. The children were aged from 3 years to 13 years and their estimated glomerular filtration rate varied from 8 to 13 ml/min per 1.73 m² when ARCD was diagnosed. Their primary renal disorders, which included hemolytic-uremic syndrome and focal segmental glomerulosclerosis, had been present for 1.5–11.5 years prior to the cysts being discovered. These patients show that ARCD may develop in children with chronic progressive renal parenchymal disease prior to the institution of specific therapy for end-stage renal disease.     

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 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 in HD: a study of 82 nephrectomies in young patients.

In order to study the development of acquired renal cystic disease (ARCD) and its potential complications, we studied, macro- and microscopically, 82 surgical specimens of nephrectomy carried out on young patients with chronic renal failure previous to renal transplantation. Statistical correlation of pathological findings with age, sex and time on hemodialysis (HD) have been done. There were 72 cases of ARCD (87.8%). It was statistically correlated with male sex (p less than 0.02) and prolonged time on HD (p less than 0.001) as has been previously reported. Hyperplasia of the cystic epithelium was found in 42 cases (52%), with 18 (22%) showing marked papillary proliferation. Also, there were 22 cases (27%) with renal adenomas. This incidence of hyperplastic and neoplastic proliferations, more than would be common in such a young population (males: 33.5 +/- 9.3 years; females: 35.4 +/- 11.7 years), suggests the potential of patients affected by ARCD to develop neoplasms. Thus, we consider that these patients must be checked periodically to detect possible malignant neoplasms.     

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 the kidneys in chronic hemodialyzed and renal transplant patients

In this study, the appearance of renal cysts in 43 chronic hemodialyzed patients (mean duration of dialysis treatment: 26.3 months) was investigated by sonography with a high resolution (3.5 MHz) sector scanner. In an investigation of the patients' own kidneys, 23 renal transplant patients (mean observation time: 51.3 months after transplantation; mean dialysis treatment before transplantation: 22.8 months) were also studied by sonography. Cysts could be demonstrated in 21 of 43 (49%) patients on maintenance hemodialysis. In 10 of these patients a previous investigation at the beginning of dialysis did not demonstrate any cysts. The diameter of the cysts varied between 5 and 30 mm. With regard to the duration of dialysis, cysts could be demonstrated by sonography in 9 of 23 (39%) dialyzed patients, with a maximum dialysis duration of 2 years, and in 12 of 20 (60%) patients who had been dialyzed for more than 2 years. The development of acquired cystic disease of the kidneys in dialysis patients seems to be promoted by the longer survival of uremic patients. Cysts could be found in the patients' own kidneys in only 4 of 23 (17%) renal transplant patients. The difference in the demonstration of cysts between patients on maintenance hemodialysis and renal transplant patients was statistically significant. This suggests that cystic transformation may possibly be a reversible process.     

Unilateral renal cystic disease in adults.

Unilateral renal cystic disease (URCD) is morphologically indistinguishable from autosomal dominant polycystic kidney disease (ADPKD) except for its unilaterality. Unlike ADPKD, URCD patients show neither a genetic background nor progressive deterioration in renal function; thus, the differential diagnosis of URCD from ADPKD is important. Only a few cases of URCD have been reported. This study reports two cases of URCD in adults together with a literature review. We identified these two cases using abdominal computerized tomography and family screening with renal ultrasonography.