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 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 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.     

Clinical evaluation of renal cell carcinoma and acquired cystic disease of the kidney in long-term dialysis patients

Long-term dialysis patients frequently develop acquired cystic disease of the kidneys (ACDK). One hundred and sixty-seven dialysis patients were classified into two groups according to the presence or absence of ACDK, and the two groups were compared with regard to the dialysis period, laboratory findings, and so forth. Among the 167 dialysis patients, 37.7% showed ACDK and had an average dialysis duration of 10.3 years, which was significantly longer than the 5.0 years for patients without ACDK. The proportion of patients with ACDK rose with increasing duration of dialysis; 64.6% of the patients on dialysis for more than 10 years had ACDK. The values of hemoglobin and hematocrit of patients with ACDK were significantly higher than those without ACDK. Of 12 dialysis patients who were operated upon because of suspected renal tumor, pathological examination confirmed renal cell carcinoma in 8 and adenoma in one. One of these patients had been treated with chronic ambulatory peritoneal dialysis, and another had bilateral renal tumor. Among the 8 patients with renal cell carcinoma, ACDK was detected in 5 by means of CT and ultrasound tomography, and cystic changes were observed in two on light microscopic examination of the resected kidneys. Pathological examination of the non-tumorous renal tissues revealed hyperplastic or adenomatous changes in the cyst epithelium in 5 patients undergoing dialysis for more than 7.5 years. These changes were thought to be pre-neoplastic. The high incidence of ACDK and the development of renal tumors in long-term dialysis patients indicates the necessity of intensive monitoring.     

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 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.     

长期血透患者获得性囊性肾病合并肾癌8例报告并文献复习

目的:学习长期血透患者获得性囊性肾病合并肾癌的筛查和诊治方法。方法:回顾性分析我院维持性血透获得性囊性肾病合并肾癌患者8例,均为B超和CT诊断为双肾多发性囊肿合并肾实质性占位,并行后腹腔镜下根治性肾切除术,术后维持规律性血透,并严密随访。结果:长期血透患者226例,获得性囊性肾病105例(46.5%),获得性囊性肾病合并肾癌8例(3.5%),在获得性囊性肾病中发生率为7.6%(8/105),其中男5例,女3例,年龄(58.6±16.4)岁,血透(12.2±6.9)年。8例患者(9次)行后腹腔镜下根治性肾切除术,手术均成功,出血(45.2±20.3)ml,手术时间(72.5±20.3)min,无严重手术并发症,术后病理3例为透明细胞癌和6例为乳头状癌。住院天数为(7.5±2.4)d。随访12～63个月,无瘤存活5例。结论:肾癌在获得性囊性肾病患者中发病率高,随着血透患者寿命的延长,血透3年后需重视和建立肾癌筛查机制,腹腔镜下根治性肾切除术安全有效、恢复快,并注重患者心脑血管疾病及糖尿病等并发症的积极治疗,有助于进一步延长血透患者寿命。     

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.     

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 in chronically rejected renal transplants.

Acquired cystic disease has been documented to complicate most forms of chronic renal damage; it has only infrequently been described in transplanted kidneys. Five patients with noncystic ESRD and chronically rejected transplants in which acquired cystic disease arose are reported. The diagnosis of acquired cystic disease was established in examination of transplant nephrectomies from four patients and a core biopsy from the fifth. The allografts were in place from 44 to 80 months; three patients were treated with hemodialysis before the diagnosis of acquired cystic disease, whereas two received peritoneal dialysis. Three of the four patients evaluated had cysts in the native kidneys. Although papillary hyperplasia of lining epithelium was evident in four specimens, only one kidney was the site of neoplasms in the form of multiple small tubular adenomas. No malignant neoplasms were noted in this study or in the few similar previous ones; however, it is possible that chronically rejected transplanted kidneys may harbor neoplasms with the same malignant potential as those in acquired cystic disease in native kidneys.     

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 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.     

Acquired cystic disease of the kidneys and renal cell carcinoma in chronic renal insufficiency without dialysis treatment

We report 3 cases of acquired cystic disease of the kidneys with associated renal carcinoma in 2 of the cases. In all 3 cases, the patients had chronic renal insufficiency due to hypertension but had never required dialysis. Review of 176 reported cases of acquired cystic disease of the kidneys and renal tumors disclosed that 18 patients (including 1 previously reported by us) had never received dialysis treatment. These cases support the hypothesis that acquired cystic disease of the kidney is not restricted to patients treated with maintenance dialysis. Among the 18 patients, hypertension was the most common underlying cause of renal failure. Patients with chronic renal failure due to or associated with severe hypertension should be monitored carefully for the development of both renal cysts and tumors even though they have not started on chronic dialysis.     

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.