Renal cell carcinoma in acquired cystic disease of the kidney manifested by spontaneous renal rupture

A 67-year-old male had been maintained on hemodialysis for 13 years because of chronic renal failure secondary to diabetes mellitus. The patient was referred to our hospital with sudden right flank pain. Computed tomography revealed multiple cysts in both kidneys and a right massive perirenal hematoma. Although there was no definite evidence of a renal tumor, a right nephretomy was performed. Histological study revealed acquired cystic disease of the kidney (ACDK) with a hematoma containing papillary renal cell carcinoma. He has been free of recurrence for 2 months. To our knowledge, this case is the fourth report of renal cell carcinoma in ACDK manifested by spontaneous rupture in the Japanese literature.     

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 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.     

Nontraumatic subcapsular renal hematoma: report of a case

A case of nontraumatic subcapsular renal hematoma was reported. The patient was a 41-year-old woman. She complained of right flank colic pain. Her excretory pyelography showed right hydronephrosis and ureteral stone, and demonstrated a large mass involving the right kidney and medial displacement of its collecting system. Ultrasonic examination and computed tomography revealed right subcapsular renal hematoma. Since we found no malignancy, we made a puncture and drained the lesion. A bloody aliquot was gained and its cytological examination was negative. Her clinical course was uneventful. After 6 months the hematoma was absorbed clearly. A review was made of 38 cases of nontraumatic subcapsular renal hematoma, including our own case. Of these, the cause was unidentified in 17 cases. Hydronephrosis, mostly associated with calculi, renal infarcts, nephritis and renal cell carcinoma followed. Of 38 cases, 2 (5.3%) were of malignant tumor.     

Ultrasonographic study on kidneys in patients with chronic renal failure. Part II. Acquired cystic disease of the kidneys

Ultrasonic examination of the kidney was performed on 280 patients undergoing chronic dialysis. Acquired cystic disease of the kidney (ACDK) was detected in 107 of 529 kidneys (20.2%). This paper presents an analysis of ultrasonotomograms of ACDK. Ultrasonic measurement of the size of ACDK was 72.5 +/- 15.2 mm in length and 41.7 +/- 9.8 mm in thickness. The size of ACDK was significantly greater than that of contracted kidneys by ultrasonographic diagnosis. With regard to sex distinction the length and thickness of ACDK were significantly greater in males than in females. As for laboratory data, patients with ACDK showed significantly higher values of red blood cell count, hematocrit and serum creatinine concentration compared with contracted kidneys. Prolongation of the dialysis peirod increased the incidence of ACDK. The size of ACDK showed a tendency to increase with duration of dialysis. However, no correlation was noted statistically between the incidence of ACDK and duration of dialysis and between the size of ACDK and duration of dialysis. There was a significantly lower incidence of ACDK in patients with diabetic nephropathy than those with chronic glomerulonephritis. A sonographic feature of ACDK is irregularity of the renal contour because of cystic transformation. Renal imaging, identification of the corticomedullary border, identification of the central echoes and increased parenchymal echogenicity were similar to other dialyzed kidneys. The main complications of ACDK are hemorrhage and tumor formation. We observed two retroperitoneal hematomas and one renal cell carcinoma developed within two years after this examination. The incidence of complications of ACDK was 5.1 per cent. We believe that patients with ACDK should be watched carefully by regular ultrasonic examination for early diagnosis and treatment of these complications.     

Renal neoplasias in patients receiving dialysis and renal transplantation: clinico-pathological features and p53 gene mutations.

BACKGROUND: In Japan, the relative risk for renal cell carcinoma (RCC) in renal transplants was about 80-fold higher than that in the general population. Depressed immune surveillance due to the use of immunosuppressive agents was considered to cause cancer. Before renal transplantation, a vast majority of patients received hemodialysis, a known causative factor for acquired cystic disease of kidney (ACDK). Because ACDK is also considered to predispose to RCC, at least two risk factors for cancer accumulate in renal transplants. METHODS: In our study, clinicopathological features together with p53 gene mutations were analyzed in 218 patients with RCC: 22 received dialysis followed by renal transplantation, 39 received dialysis alone, and 157 sporadic RCC. P53 mutations were analyzed on DNA extracted from paraffin-embedded specimens with use of single strand conformation polymorphism, followed by direct sequencing. RESULTS: RCC in transplants shared several clinicopathological features with those in dialysis patients, which included small size and multiplicity of tumor, relatively high frequency of presence of ACDK, and papillary type of RCC. p53 gene mutations were infrequent in RCC of any clinical setting. CONCLUSIONS: Atrophic kidney at the end-stage of renal failure and under dialysis have lesions of ACDK that might predispose to RCC in dialysis and transplant patients.     

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.     

A case of acquired cystic disease of bilateral kidneys with renal cell carcinoma following long-term hemodialysis]

We report a case of bilateral renal cell carcinoma which developed during about 14 years of hemodialysis. The patient of male was a 39-year-old with a chief complaint of macrohematuria 14 years prior to dialysis therapy. Computed tomography revealed multiple cystic changes of bilateral kidneys and a high density area in the right kidney. He was admitted to our department in April 1988. He was suspected of renal cell carcinoma of the right kidney and underwent transperitoneal radical nephrectomy on the right kidney. The left kidney was also resected simultaneously in part because it failed to function and in part because the long-term hemodialyzed patients are considered to have a complication of renal cell carcinoma at a high frequency. Pathological diagnosis was bilateral acquired cystic disease of the kidney (ACDK) with renal cell carcinoma. The kidney develops multiple cystic changes following long-term hemodialysis, which forms a high risk for development of renal cell carcinoma. This is a case of bilateral renal cell carcinoma which occurred after long-term hemodialysis, was reported with discussion and reference to the literature.     

Renal cell carcinoma in a long-term hemodialysis patient: a case report of its interesting clinical course

A 61-year-old man with a history of hemodialysis for 10 years, and complaining of fever and right flank pain was introduced to us with the suspicion of right renal tumor and admitted on May 19, 1984. Right radical nephrectomy was done and the histological diagnosis was renal cell carcinoma (clear cell type) with acquired cystic disease of the kidney (ACDK). On the 27th day after operation, spontaneous rupture of bladder, despite its lack in functioning was suspected and an emergency operation was done. The bladder wall was very thin and weak and it was lacerated about 5 cm. After this operation the blood pressure was unstable and the patient died on the 64th day after nephrectomy due to sepsis. In Japan, 9 cases (containing this case) of renal cell carcinoma associated with ACDK were reported. The mean age and the mean duration of dialysis were 37.4 years old and 6.7 years. The cause of bladder rupture (1973-1983) in 87 cases is discussed.     

Xanthogranulomatous pyelonephritis with acquired cystic disease of the kidney in a haemodialysis patient

SUMMARY:   A 54-year-old-female patient who had received regular haemodialysis therapy for 12 years was referred to our hospital for evaluation of a left renal mass. Imaging examinations revealed acquired cystic disease of the kidney (ACDK) and a tumour-like lesion in the left kidney. Because of the preoperative diagnosis of the left renal cell carcinoma, the patient underwent a left nephrectomy. Pathological examination revealed xanthogranulomatous pyelonephritis. It was difficult to distinguish xanthogranulomatous pyelonephritis from renal cell carcinoma in our case, because it is very rare for xanthogranulomatous pyelonephritis to occur in ACDK.     

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 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.     

Chromophobe cell renal carcinoma with acquired cystic disease of the kidney in a long-term hemodialysis patient

We report a rare case of chromophobe cell renal carcinoma found in a 52-year-old female who had received hemodialysis therapy for 13 years. She was diagnosed as having a left renal tumor 7.5 cm in diameter with acquired cystic disease of the kidney (ACDK) by ultrasonographic examination during periodical systemic screening. As abdominal computed tomography scanning and enhanced color Doppler ultrasonography suspected that the hypervascular tumor was renal cell carcinoma, she underwent translumbar nephrectomy in July 2000. The histopathological diagnosis was chromophobe cell carcinoma with pT2 and grade 2 malignancy. Chromophobe cell carcinoma is uncommon among renal tumors with ACDK found in long-term hemodialysis patients.     

Kidney rupture: an unusual and oligosymptomatic complication in a dialysis patient with acquired cystic disease

Acquired cystic kidney disease (ACKD) is a complication of end-stage renal disease, the prevalence of which is related to dialysis duration; incidence of ACKD and associated conditions (neoplasia, hemorrhage) have decreased with improvements in renal transplantation and with the ageing of the dialysis population. This report regards spontaneous kidney rupture in a 57-year old patient, on home hemodialysis for 11 years, with ACKD for 5 years. At the end of a dialysis session, the patient reported sudden onset of colicky flank pain, followed by macrohematuria. Pain remitted with low doses of pain relievers, leaving dull flank discomfort. The patient self diagnosed a renal colic, and called the hospital two days later. At referral, two large hemorrhagic renal masses (7 and 2.8 cm) were found at ultrasound and CT scan. At surgery, kidney rupture was diagnosed. This case highlights the life threatening complications associated with ACKD, and underlines that massive renal hemorrhage may occur with relatively minor symptoms.     

CONSERVATIVE MANAGEMENT OF NON-TRAUMATIC SUBCAPSULAR RENAL HEMATOMA: A CASE REPORT

The case of a 78-year-old woman with a non-traumatic subcapsular renal hematoma is described. She complained of right flank pain and fever and the findings following abdominal echography, computerized tomography (CT) scan and renal angiography agreed with a diagnosis of subcapsular renal hematoma without renal tumor. Percutaneous drainage of the hematoma improved her clinical symptoms and the lesion as seen on CT scan. Ten months later, no change in the right kidney could be seen on CT scan and echography. We conclude that conservative management is the first choice for subcapsular renal hematoma without a definite etiology, especially in an elderly patient.     

Two cases of renal cell carcinoma arising in the native kidney following renal transplantation--clinical study and review of 26 cases reported in Japan

Renal transplant recipients have an increased risk of developing malignancies due to long-term immunosuppression. Two cases of renal cell carcinoma arising from the native kidney following renal transplantation are reported. Case 1; A 60-year-old man underwent cadaveric renal transplantation on July 31, 1995. He had resumed hemodialysis because of graft failure on June 5, 2000. He was referred to our hospital with macroscopic hematuria on March, 2002. Computed tomography (CT) demonstrated a right renal mass of about 3.0 cm in diameter with bilateral acquired cystic disease of the kidney (ACDK). The radical nephrectomy was performed. Case 2; A 55-year-old man underwent cadaveric renal transplantation on November 15, 1996. He had resumed hemodialysis because of graft failure on June 5, 1996. A left renal mass measuring 3.0 cm in diameter as well as bilateral ACDK were found by CT on July, 2002. Left radical nephrectomy was performed. The histological diagnosis of both renal tumors was renal cell carcinoma, clear cell type, G2, pT1a. Fifty-six renal transplantations (48 were renal allografts from cadavers, while 8 were from living donors) were performed in our hospital from 1980 to 2002. Two patients (3.6%) developed renal cell carcinoma after transplantations. We also discuss a clinical study and review of 26 cases reported in Japan. Since the incidence of malignant neoplasia is high, CT and ultrasonography should be performed routinely on the patients with renal transplantation.     

Somatic mutations of the von Hippel-Lindau disease gene in renal carcinomas occurring in patients with long-term dialysis.

BACKGROUND: Renal cell carcinoma (RCC) frequently occurs in patients with long-term dialysis. Long-term dialysis causes distinctive pathological changes in the kidney, which is known as acquired cystic disease of the kidney (ACDK). It is of great interest to know whether RCCs occurring in the dialytic kidneys harbour the same or similar mutations of the von Hippel-Lindau (VHL) gene as conventional dialysis-unrelated clear cell RCCs so often do. METHODS: Renal cancer tissues (eight clear cell, two papillary, one Bellini duct and three of the so-called dialysis-specific renal carcinomas) from 13 patients undergoing long-term dialysis were examined for somatic mutations of the VHL disease gene. By means of laser capture microdissection, cancerous and surrounding non-cancerous renal tissues from dialytic patients were subjected to PCR-based direct sequencing of the VHL gene. RESULTS: Direct forward and reverse sequencing showed that three tumours possessed VHL gene mutations (713delG, 500-504del5-bp and 709A>G). These three mutations were identified in clear cell carcinomas occurring in association with end-stage renal disease undergoing dialysis for 194, 147 and 125 months. None of the non-tumour tissues or other carcinoma tissues analysed, including dialysis-specific carcinoma, possessed VHL gene mutations. CONCLUSION: These results indicate that VHL tumour-suppressor gene mutation is involved in clear cell carcinoma in association with long-term dialysis. Mutation of the VHL gene was not found in any of the dialysis-specific RCCs studied herein.     

Comparison of ultrasonography, computerized tomography and angiography in dialysis patients with renal cell carcinoma.

Seventeen dialysis patients with renal cell carcinoma were examined preoperatively by ultrasonography, computerized tomography (CT) and angiography. Ultrasonography, CT and angiography correctly predicted the diagnosis of renal cell carcinoma in 17 (100%) and 12 (71%) of 17, and 11 (69%) of 16 patients, respectively. All renal tumors of more than 3.0 cm in diameter were detected by the three diagnostic procedures. However, ultrasonography, CT and angiography detected renal tumors of less than 3.0 cm in 12 (100%) and 7 (58%) of 12 patients and 6 (55%) of 11 patients, respectively. Seven renal cell carcinomas were associated with acquired cystic disease of the kidney (ACDK). All renal tumors were found by ultrasonography. However, CT predicted the correct diagnosis in 3 (43%) of 7 patients with ACDK and in 9 (90%) of 10 patients without ACDK, and angiography was correct in 3 (50%) of 6 patients with ACDK and 8 (80%) of 10 patients without ACDK. Our results indicate that ultrasonography is an effective procedure to detect renal cell carcinomas in dialysis patients.     

化学诱导大鼠多发肾囊肿伴肾肿瘤及肾组织DNA损伤的表达

目的 观察化学药物大鼠多发肾囊肿伴肾肿瘤模型的诱发情况和ＤＮＡ损伤标记物８－羟基脱氧鸟苷（８－ＯＨｄＧ）在其肾组织的表达。方法 用２－氨基４,５－二苯噻唑（ＤＰＴ）和Ｎ－亚硝基吗啉（ＮＮＭ）诱发和人类获得性肾囊肿（ＡＣＤＫ）伴肾癌相似的大鼠动物模型,并用免疫组化方法（ＬＳＡＢ法）检查８－ＯＨｄＧ在大鼠肾组织的表达。结果在本实验中,ＮＮＭ组３／１０的大鼠诱发肾肿瘤出现,在ＤＰＴ／ＮＮＭ组的９只大鼠均     

输尿管镜碎石术后肾包膜下血肿危险因素分析

目的：探讨输尿管镜碎石术后发生肾包膜下血肿的危险因素。方法：回顾性分析2006年7月～2008年12月行输尿管镜碎石术后发生肾包膜下血肿3例患者的病例资料，评估其术前、术中可能存在的导致肾包膜下血肿发生的风险因素。结果：2例行双侧输尿管镜碎石术，1例行单侧输尿管镜碎石术，手术均顺利，但均发生肾包膜下血肿，其中1例术前并有高血压、糖尿病、尿路感染，发生双侧肾包膜下血肿并失血性休克；1例老年男性术前无其他并发症，术后术侧腰部出现皮下瘀斑，B超发现肾包膜下血肿；另1例术前并发高血压、糖尿病，行双侧输尿管镜碎石术，术后发生单侧肾包膜下血肿。结论：肾包膜下血肿是输尿管镜碎石术的一个严重并发症，高血压、糖尿病、高龄、尿路感染是其发生的危险因素，而手术本身导致的肾盂内压力改变是其发生的诱发因素。