Collecting duct carcinoma with acquired cystic disease of the kidney in a long-term hemodialysis patient

Abstract:   We report a very rare case of collecting or Bellini duct carcinoma (CDC) found in a 60-year-old male who had received hemodialysis therapy for 21 years. Screening with ultrasonography revealed a solid tumor originating from the cyst wall in the right kidney with acquired cystic disease of the kidney. Subsequent computed tomography (CT) and angiography could not detect another renal tumor. Right radical nephrectomy was performed. The tumor detected preoperatively was composed of papillary renal cell tumor (RCC) and multiple clear cell carcinoma, pathologically. In addition to the tumors, CDC was revealed in the central medulla with the involvement of regional lymph nodes. Three months later, left nephrectomy was performed because left RCC was suspected during CT. The histological diagnosis was multiple clear cell carcinomas. Peritonitis carcinomatosa appeared and the patient died 13 months later.     

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.     

Xanthogranulomatous pyelonephritis with a renocolic fistula caused by a parapelvic cyst

Fistula formation between the upper urinary tract and bowel is an uncommon complication in urogenital diseases. We present a rare case of focal xanthogranulomatous pyelonephritis with a renocolic fistula. This is the first case where a parapelvic cyst obstructs the caliceal outflow and leads to the formation of a renocolic fistula in renal inflammatory disease. It is difficult to make a preoperative diagnosis of focal xanthogranulomatous pyelonephritis with widespread involvement that is caused by non-calculous urinary tract obstruction.     

Xanthogranulomatous pyelonephritis: presentation and management in the era of laparoscopy

OBJECTIVE

To report a contemporary series of patients with xanthogranulomatous pyelonephritis (XGP, an inflammatory condition of the kidney that has traditionally been treated with open nephrectomy, ON), managed with either ON or laparoscopic nephrectomy (LN), as early reports suggest that the benefits of LN do not extend to patients with XGP.

PATIENTS AND METHODS

The medical and procedural records of patients with pathologically confirmed XGP between December 1993 to February 2004 were reviewed retrospectively. During this period, 26 patients (mean age 43.5 years, range 17–85) had surgical management of XGP; information on the mode of presentation, surgical management, hospital course and complications were analysed.

RESULTS

Twelve (46%) and 14 (54%) patients had ON and LN, respectively. The LN group had significantly lower blood loss (P = 0.002), transfusion rates (P = 0.02), time to resumption of oral intake (P = 0.002) and length of hospital stay (P = 0.002) than the ON group. One (7%) LN was converted to ON due to failure to progress. The overall complication rates between the ON and LN groups were not significantly different (P = 0.95).

CONCLUSIONS

LN in the setting of XGP is often difficult and requires advanced laparoscopic skills. In highly selected patients this approach can be offered, with acceptable morbidity, allowing for lower blood loss and shorter convalesce times.     

Xanthogranulomatous pyelonephritis: our experience with review of published reports

BACKGROUND: The aim of this study was to analyse the cases of xanthogranulomatous pyelonephritis with review of published reports. METHODS: We retrospectively reviewed all nephrectomy specimens during February 1995 to January 2006. We found 26 cases of xanthogranulomatous pyelonephritis. We prepared a chart of these cases consisting of preoperative symptoms, laboratory findings, radiological imaging results and preoperative diagnosis. Open nephrectomy was carried out in all cases. RESULTS: Age group of the patients was 6-65 years and male : female ratio was 1.6:1. Twenty-two patients presented to us with intermittent high-grade fever, 21 with flank pain, 18 with loin mass, 2 with haematuria and 1 was detected during screening of vague gastrointestinal symptoms. Twenty-five patients had pyuria and only 10 had sterile urine culture. In all patients, only one kidney was affected. All patients had renal calculi 10-42 mm in size with bilaterally enlarged kidneys. Ipsilateral kidney was enlarged because of hydronephrosis or pyonephrosis in all cases and contralateral kidney was enlarged because of compensatory hypertrophy in 13 cases. Ipsilateral kidney had severely compromised renal function in all cases. Associated psoas abscess was present in one and tuberculosis in another. CONCLUSION: Xanthogranulomatous pyelonephritis is a relatively rare entity that is associated with obstruction, stones and infection of the urinary tract. Late presentation leads to loss of renal parenchyma. It cannot be differentiated preoperatively with renal tumours (renal cell carcinoma and Wilms' tumour), pyonephrosis, infected hydronephrosis and renal lymphoma. Nephrectomy and antibiotics are the treatment of choice.     

Xanthogranulomatous pyelonephritis: a systematic review of treatment and mortality in more than 1000 cases

Objectives

To systematically review the current demographics, treatment and mortality rate associated with xanthogranulomatous pyelonephritis (XGP) and to test the hypothesis that the weighted pooled peri-operative mortality rate will be <10%.

Methods

Searches were performed of the Cochrane, Embase and Medline databases and the grey literature for studies published during the period 1 January 2000 to 30 August 2021. Eligible studies reported cohorts of ≥10 predominantly adult patients with XGP and described either average patient age or mortality rate.

Results

In total, 40 eligible studies were identified, representing 1139 patients with XGP. There were 18 deaths, with a weighted pooled peri-operative mortality rate of 1436 per 100 000 patients. The mean age was 49 years, 70% of patients were female and 28% had diabetes mellitus. The left kidney was more commonly affected (60%). Four patients had bilateral XGP, and all of whom survived. Renal or ureteric stones were present in 69% of patients, including 48% with staghorn calculi. Urine culture was positive in 59% of cases. Fistulae were present in 8%. Correct preoperative diagnosis occurred in only 45% of patients. Standard treatment continues to comprise a short cause of antibiotics and open radical (total) nephrectomy. Preoperative decompression occurred in 56% of patients. When considered at all, laparoscopic nephrectomy was performed in 34% of patients. Partial nephrectomy was conducted in 2% of patients.

Conclusions

Xanthogranulomatous pyelonephritis has a lower mortality rate than historically reported. A typical patient is a woman in her fifth or sixth decade of life with urolithiasis. While open radical nephrectomy remains the most common treatment method, laparoscopic, and to a lesser degree partial nephrectomy, are feasible in well selected patients.     

Diffuse xanthogranulomatous pyelonephritis in a patient with myotonic dystrophy and cerebral palsy

Xanthogranulomatous pyelonephritis (XGP), a morphological and clinical variant of chronic pyelonephritis, is an uncommon disease in children. It is characterized by the destruction of the renal parenchyma and replacement by granulomatous tissue containing foamy lipid-laden macrophages and is classified into diffuse and focal XGP. We present a case of diffuse XGP in a child with myotonic dystrophy complicated by cerebral palsy and discuss the importance of correct diagnosis and preoperative management to reduce inflammation and improve malnutrition associated with the disease.     

Pilot study of non‐contrast‐enhanced MRI vs. ultrasound in renal transplant recipients with acquired cystic kidney disease: a prospective intra‐individual comparison

The incidence of renal cell carcinoma (RCC) after kidney transplantation is 15‐fold increased. Acquired cystic kidney disease (ACKD) is one of the known risk factors. We performed a small pilot study to assess the role of non‐enhanced magnetic resonance imaging (MRI) as a tool for intensified screening in renal transplant recipients with ACKD. Renal ultrasound was used to assess the native kidneys of 215 renal transplant recipients. Thirty patients with 54 kidneys, fulfilling the criteria of ACKD, underwent non‐enhanced MRI at 1.5T using T2‐ and T1‐weighed as well as diffusion‐weighted sequences with a high spatial resolution. Among the 54 kidneys assessed by both methods, three RCCs were identified (6%). Of those, one RCC was detected by both imaging methods (33%), while two RCCs were diagnosed by MRI alone (67%). MRI identified an additional four proteinaceous or hemorrhagic cysts that did not fulfill the criteria for RCC but were classified as suspicious. All of these lesions were stable in size and appearance in follow‐up studies. In conclusion, non‐enhanced MRI was more sensitive than ultrasound in identifying RCCs and lesions suspicious for RCC and thus appears to be a useful secondary screening tool in patients with ACKD after renal transplantation.     

Unilateral and segmental cystic disease of the kidney

Unilateral and segmental cystic disease is a rare cystic disease of the kidney. Except for its unilaterality, it resembles autosomal dominant polycystic kidney disease on gross and histological examination. It also lacks genetic background and progressive deterioration of renal function. Only a small number of unilateral and segmental cystic disease of the kidney cases have been reported to date. The present case is a report of a 69-year-old man who had a large multicystic mass involving the lower and middle part of the right kidney with normal residual renal parenchyma at the upper pole. The left kidney was entirely normal. All the laboratory tests were within normal limits. Right nephrectomy was performed because of a strong suspicion of malignancy.     

Effect of treatment mode on the natural history of acquired cystic disease of the kidney in patients on renal replacement therapy.

The natural history of acquired cystic disease of the kidney has been investigated in a long-term follow-up study of patients on renal replacement therapy. A cohort of 145 end-stage renal failure patients was initially investigated with ultrasonography to determine the degree of cystic change. Seventy-three patients were available for follow up a minimum of 3 years later. The grade of cystic disease progressed in dialysis patients and progression was more marked in haemodialysis patients than patients maintained on CAPD. Patients with functioning renal transplants did not show progression of cystic change and in two patients regression was seen. Nine patients maintained on chronic dialysis at the time of initial ultrasound subsequently received renal grafts, and three of these patients had evidence of regression of cystic change on follow-up scanning. After 3 years follow-up a single haemodialysis patient had evidence of a solid lesion in a cystic kidney and this has not progressed during a further 12 months of follow-up. Acquired cystic disease of the kidney is a progressive disease in chronic dialysis patients. However, over a follow-up period of 3 years, patients with functioning renal grafts do not show similar progression. The incidence of solid renal tumours has been shown to be low.     

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。     

Polysomnographic measures of nocturnal sleep in patients on chronic, intermittent daytime haemodialysis vs those with chronic kidney disease.

BACKGROUND: Numerous factors probably contribute to the high prevalence of sleep problems in haemodialysis (HD) patients including metabolic changes and treatment-related factors. In contrast, the sleep problems of patients with chronic kidney disease (CKD) may be more related to psychological factors rather than the metabolic changes associated with renal disease. Thus, the objective of this study was to compare polysomnographic measures of nocturnal sleep in a group of stable patients on chronic, intermittent daytime HD and an age- and gender-matched, metabolically comparable group with CKD, and evaluate the role that quality of life (including psychological factors) and the effects of treatment may play in sleep outcomes. METHODS: The sample included 16 patients on HD and eight patients with CKD all of whom were free from other significant physical and psychological morbidity. To assess for psychological, functional, family and economic responses to the disease and treatment, all subjects took the Ferrans and Powers Quality of Life Index. HD subjects received treatment three times a week and were adequately dialysed [Kt/V >1.2, equivalent to a weekly glomerular filtration rate (GFR) of 10-15 ml/min]; CKD subjects had an estimated GFR of 14.5 (+/-7.2; range 5.4-28.8) ml/min. All subjects underwent one night of laboratory-based polysomnography. Appropriate statistical procedures were used to explore group differences in sleep variables and their relationship to quality of life dimensions and the effect of treatment. RESULTS: The CKD patients reported significantly poorer functional and psychological quality of life; both groups had reduced total sleep time and sleep efficiency in comparison with normative data. However, HD subjects had less rapid eye movement sleep (P = 0.032). They also had a higher brief arousal index (P = 0.000), an independent predictor of which was treatment with HD, and respiratory disturbance index (P = 0.061). Less total sleep time, increased wake after sleep onset, lower sleep efficiency, higher periodic limb movement index, and longer latencies to sleep onset and rapid eye movement sleep were also noted in the HD group. Quality of life scores did not predict sleep variables in this small sample. CONCLUSIONS: The results suggest that the sleep problems of patients with CKD and those receiving chronic, intermittent daytime HD may have different aetiologies; functional and psychological factors may play a more prominent role in the former group, while intrinsic sleep disruption (arousals, apnoeas and limb movements) secondary to the effects of chronic, intermittent daytime HD may play a more significant role in the latter. The findings suggest that further exploration is warranted and that population-specific sleep-promoting interventions may be indicated.     

Ankle brachial index as a predictor for mortality in patients with chronic kidney disease and undergoing haemodialysis

Aim: The ankle brachial index (ABI) is a marker for peripheral artery disease and can predict mortality in advanced chronic kidney disease (CKD) and haemodialysis patients, respectively. However, it is seldom studied in Taiwan, an area with high prevalence of CKD and end‐stage renal disease. The aim of this study was to investigate the predictors for mortality by using ABI value in patients with CKD and undergoing haemodialysis in Taiwan. Methods: One hundred and sixty‐nine patients with CKD stage 3–5 and 231 haemodialysis patients were enrolled in one regional hospital. The mean follow‐up period was 23.3 ± 3.3 months. Patients were stratified into three groups according to ABI value (<0.9, ≥0.9 to <1.3, and ≥1.3). The relative mortality risk was analyzed by Cox‐regression methods. Results: In multivariate analysis, an ABI of 1.3 or more (hazard ratio, 3.846; P = 0.043) and coronary artery disease (P = 0.012) were positively associated with overall mortality, and serum low‐density lipoprotein cholesterol level (P = 0.042) was negatively associated with overall mortality. In addition, an ABI of less than 0.9 (P = 0.049), an ABI of 1.3 or more (P = 0.033), coronary artery disease (P = 0.024) and haemodialysis treatment (P = 0.043) were strong predictors for cardiovascular mortality. Conclusion: Our findings show that an ABI of 1.3 or more predicts for both overall and cardiovascular mortality, and an ABI of less than 0.9 predicts for cardiovascular mortality in CKD and haemodialysis patients. Screening patients with chronic renal failure by means of ABI may help to identify a high‐risk group for increased mortality.     

New insights: nephronophthisis-medullary cystic kidney disease

Nephronophthisis (NPH) and medullary cystic kidney disease (MCKD) constitute a group of renal cystic diseases, which share a common characteristic renal histologic triad of tubular basement membrane disintegration, tubular atrophy with cyst development, and interstitial cell infiltration with fibrosis. The different disease variants lead to chronic renal failure with onset at characteristic age ranges for recessive NPH and dominant MCKD. There is extensive gene locus heterogeneity with at least three different loci for nephronophthisis (NPHP1, NPHP2, and NPHP3) and two different loci for MCKD (MCKD1 and MCKD2). Juvenile nephronophthisis, in addition, can be associated with extrarenal organ involvement. We have identified by positional cloning the gene (NPHP1) for juvenile nephronophthisis (NPH1), as a first step towards understanding the pathogenesis of this disease group. Its gene product, nephrocystin, is a novel protein, which contains a src-homology 3 (SH3) domain. We put forward a hypothesis that the pathogenesis of NPH might be related to signaling processes at focal adhesions (the contact points between cells and extracellular matrix) and/or adherens junctions (the contact points between cells). Received: 13 July 2000 / Revised: 1 October 2000 / Accepted: 4 October 2000     

Three-dimensional architecture of nephrons in the normal and cystic kidney

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