Acquired Cystic Kidney Disease Research Articles

Incidental finding of acquired cystic kidney disease associated renal cell carcinoma in an adolescent with kidney failure.

Acquired cystic kidney disease (ACKD) can occur in patients with chronic kidney disease and kidney failure, and its incidence increases with the duration of dialysis. In adults, ACKD is less common in the pre-dialysis group (~ 7%), but its incidence can be as high as 80% for those who are on dialysis for more than ten years. There is, however, very little information about the prevalence of ACKD in children. We report a case of malignant transformation of ACKD following a kidney transplant, highlighting the importance of surveillance of the native kidneys in paediatric patients who have been in long-term kidney replacement therapy.

Wall Tension and Tubular Resistance in Kidney Cystic Conditions.

The progressive formation of single or multiple cysts accompanies several renal diseases. Specifically, (i) genetic forms, such as adult dominant polycystic kidney disease (ADPKD), and (ii) acquired cystic kidney disease (ACKD) are probably the most frequent forms of cystic diseases. Adult dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by multiple kidney cysts and systemic alterations. The genes responsible for the condition are known, and a large amount of literature focuses on the molecular description of the mechanism. The present manuscript shows that a multiscale approach that considers supramolecular physical phenomena captures the characteristics of both ADPKD and acquired cystic kidney disease (ACKD) from the pathogenetic and therapeutical point of view, potentially suggesting future treatments. We first review the hypothesis of cystogenesis in ADPKD and then focus on ACKD, showing that they share essential pathogenetic features, which can be explained by a localized obstruction of a tubule and/or an alteration of the tubular wall tension. The consequent tubular aneurysms (cysts) follow Laplace's law. Reviewing the public databases, we show that ADPKD genes are widely expressed in various organs, and these proteins interact with the extracellular matrix, thus potentially modifying wall tension. At the kidney and liver level, the authors suggest that altered cell polarity/secretion/proliferation produce tubular regions of high resistance to the urine/bile flow. The increased intratubular pressure upstream increases the difference between the inside (Pi) and the outside (Pe) of the tubules (∆P) and is counterbalanced by lower wall tension by a factor depending on the radius. The latter is a function of tubule length. In adult dominant polycystic kidney disease (ADPKD), a minimal reduction in the wall tension may lead to a dilatation in the tubular segments along the nephron over the years. The initial increase in the tubule radius would then facilitate the progressive expansion of the cysts. In this regard, tubular cell proliferation may be, at least partially, a consequence of the progressive cysts' expansion. This theory is discussed in view of other diseases with reduced wall tension and with cysts and the therapeutic effects of vaptans, somatostatin, SGLT2 inhibitors, and potentially other therapeutic targets.

Renal Cell Carcinoma in End-Stage Renal Disease: A Retrospective Study in Patients from Hungary

Introduction: End-stage renal disease (ESRD) and acquired cystic kidney disease (ACKD) are known risk factors for renal cell carcinoma (RCC). Hereby, the clinicopathological features of RCCs developed in ESRD were investigated. Methods: A database consisting of 34 tumors from 31 patients with ESRD among 2,566 nephrectomy samples of RCC was built. The demographic, clinical, and follow-up data along with pathological parameters were analyzed. The RCCs were diagnosed according to the current WHO Classification of Urinary and Male Genital Tumors. Results: Twenty-two tumors developed in men and 12 in women, with a median age of 56 years (range: 27–75 years). The causes of ESRD were glomerulonephritis (n = 7), hypertensive kidney disease (n = 6), autosomal dominant polycystic kidney disease (n = 6), chronic pyelonephritis (n = 4), diabetic nephropathy (n = 3), chemotherapy-induced nephropathy (n = 1), and undetermined (n = 4). ACKD complicated ESRD in 12 patients. The following histological subtypes were identified: clear cell RCC (n = 19), papillary RCC (n = 5), clear cell papillary tumor (n = 5), ACKD RCC (n = 3), and eosinophilic solid and cystic RCC (n = 2). The median tumor size was 31 mm (range: 10–80 mm), and 32 tumors were confined to the kidney (pT1-pT2). There was no tumor-specific death during the period of this study. Progression was registered in 1 patient. Conclusion: In our cohort, the most common RCC subtype was clear cell RCC (55%), with a frequency that exceeded international data appreciably (14–25%). The incidence of clear cell papillary tumor and ACKD RCC (14.7% and 8.5%) was lower than data reported in the literature (30% and 40%). Our results indicate a favorable prognosis of RCC in ESRD.

Cystic Kidney Diseases That Require a Differential Diagnosis from Autosomal Dominant Polycystic Kidney Disease (ADPKD).

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary cystic kidney disease, with patients often having a positive family history that is characterized by a similar phenotype. However, in atypical cases, particularly those in which family history is unclear, a differential diagnosis between ADPKD and other cystic kidney diseases is important. When diagnosing ADPKD, cystic kidney diseases that can easily be excluded using clinical information include: multiple simple renal cysts, acquired cystic kidney disease (ACKD), multilocular renal cyst/multilocular cystic nephroma/polycystic nephroma, multicystic kidney/multicystic dysplastic kidney (MCDK), and unilateral renal cystic disease (URCD). However, there are other cystic kidney diseases that usually require genetic testing, or another means of supplementing clinical information to enable a differential diagnosis of ADPKD. These include autosomal recessive polycystic kidney disease (ARPKD), autosomal dominant tubulointerstitial kidney disease (ADTKD), nephronophthisis (NPH), oral-facial-digital (OFD) syndrome type 1, and neoplastic cystic kidney disease, such as tuberous sclerosis (TSC) and Von Hippel-Lindau (VHL) syndrome. To help physicians evaluate cystic kidney diseases, this article provides a review of cystic kidney diseases for which a differential diagnosis is required for ADPKD.

Acquired cystic disease subtype renal cell carcinoma (ACD-RCC): prevalence and imaging features at a single institution.

Acquired cystic kidney disease (ACKD) is commonly seen in patients with end-stage renal disease (ESRD), and patients with ACKD have an increased risk of renal cell carcinoma (RCC). Acquired cystic disease-associated RCC (ACD-RCC) was incorporated into the 2016 World Health Organization Classification. This study aims to describe the imaging features of ACD-RCC, which are not well reported previously. Retrospective review of patients with ACKD who underwent total nephrectomy for concern of a renal mass between 2016 and 2021 yielded 122 nephrectomies in 107 patients. Pathology reports were searched for type and subtype of mass. In ACD-RCC subtypes, imaging studies were evaluated for modality and contrast enhancement (CE). Imaging findings assessed included cystic/solid nature, unenhanced CT (NECT) attenuation, enhancement characteristics [non-enhancing (< 10 HU difference), equivocal (10-20 HU), enhancing (> 20 HU)], subjective MRI enhancement, T1 and T2 signal intensity, restricted diffusion, ultrasound (US) echogenicity, and subjective CEUS enhancement. 148 masses were identified, 122 (82%) of which were malignant and 26 (18%) benign. The three most common tumors were clear cell RCC (n = 47), papillary RCC (n = 35), and ACD-RCC (n = 21). Of the 21 cases of ACD-RCC, 16 had preoperative imaging: CT (15: 6 NECT only, 2 CECT only, 7 combined NECT and CECT), MRI (4), CEUS (5). Ten of these tumors were solid/mostly solid and 6 mixed cystic/solid. On NECT, the average attenuation was 35 HU (range 13-52). Of those with multiphasic CTs, 1 was non-enhancing, 3 were equivocal, and 3 enhanced. All 3 masses imaged with CE-MRI showed enhancement. All 4 tumors evaluated by MRI demonstrated T2 hypointensity and restricted diffusion. All five masses enhanced on CEUS. ACD-RCC subtype was the third most common renal neoplasm in ACKD patients. Our findings found that no single imaging feature is pathognomonic for ACD-RCC. However, ACD-RCCs are typically solid masses with most demonstrating equivocal or mild enhancement on CT. T2 hypointensity and restricted diffusion were the most common MRI features.

Acquired Cystic Kidney Disease: A Hidden Complication in Children on Chronic Hemodialysis.

ObjectiveTo determine the frequency of acquired cystic kidney disease (ACKD) in children on chronic hemodialysis.Material and methodsIn this single-center cross-sectional study, 150 children were included who were on chronic hemodialysis for six months. Ultrasound was done to see the renal cysts. Cystic changes that could not fulfill the criteria for ACKD were also noted and analyzed.ResultsThe mean age was 14.5 ± 3.5 years, of these 63 (42%) were males. Acquired cysts were detected in 53 (35%) of the patient and 18 patients (12%) had solitary cysts. The distribution of these entities was similar across all age groups. The underlying etiologies in the descending order were unknown 64 (43%), stone disease 31 (21%), each of the congenital anomalies of the kidney and urinary tract, and glomerulonephritis 23 (15%), and others nine (6%). A higher frequency of ACKD was detected in the children on renal replacement therapy for more than two years (33 out of 53 children, 63% with a p-value of 0.004).ConclusionThe ACKD was found in one-third of our hemodialysis children and its frequency increases with the duration of hemodialysis. This percentage may not reflect the true prevalence as there is a lack of consensus on the definition of ACKD. Periodic assessment of chronic kidney disease patients for the development of ACKD especially on chronic hemodialysis is required to reduce the morbidity.

POS-816 DE NOVO KIDNEY ALLOGRAFT RENAL CELL CARCINOMA: A RARE UNSALVAGEABLE CAUSE OF ALLOGRAFT LOSS IN A VERY LATE POST-TRANSPLANT PERIOD

Renal cell carcinoma (RCC) is a common malignancy in end-stage kidney disease (ESKD) from acquired cystic kidney disease (ACKD). With a net state of immunosuppression, kidney transplant recipients (KTR) are even at higher risk for RCC; however, RCC in the kidney allograft is uncommon unless it results from donor-derived disease. We report a case of old-aged woman with kidney allograft masses at the very late post-transplant period. Explanted transplant allograft revealed RCC.

Renal artery embolization for spontaneous hemorrhage in patients with acquired cystic kidney disease: A 20-year single-center experience

Objectives:To evaluate the safety and effectiveness of transcatheter arterial embolization for controlling spontaneous hemorrhage in patients with acquired cystic kidney disease (ACKD).Methods:This retrospective study included 18 patients with ACKD (M:F=13:5; mean age, 56 years) who underwent renal artery embolization to control spontaneous hemorrhage between January 2001 and September 2020. The underlying etiology and clinical presentations were reviewed and previous computed tomography (CT) findings were analyzed. Furthermore, angiographic and embolization details, technical and clinical successes, and complications were assessed.Results:Subcapsular, perirenal, and pararenal hematomas were observed on CT scans for all patients. Contrast extravasation was observed in 15 / 17 patients (88%) on contrast-enhanced CT scans. Angiography showed active bleeding in 14 patients (78%; contrast extravasation [n=6], pseudoaneurysm [n=3], and both [n=5]), suspicious bleeding in 1 (5%), and no bleeding in 3 (17%). The technical and clinical success rates were 100% and 94% (17/18), respectively. Total and partial embolization was performed in 14 (78%) and 4 (22%) cases, respectively. Subsequent surgical nephrectomy was required for one patient with clinical failure due to recurrent bleeding despite total embolization. Procedure-related complications included mild post-embolization syndrome in one patient and contrast-induced nephropathy in five patients (28%) without long-term complications.Conclusions:Renal artery embolization is safe and effective for controlling spontaneous renal hemorrhage in patients with ACKD.

Почечно-клеточный рак у пациентов, находящихся на лечении гемодиализом

Introduction. In patients with end-stage chronic renal failure (ESRD), renal cell carcinoma (RCC) is detected more often than in the general population. In order to improve the results of treatment of this category of patients, we conducted a retrospective analysis of our own experience. Materials and methods. In the period from 2010 to 2020, 26 patients undergoing hemodialysis underwent a nephrectomy for RСС in the State hospital № 52, Moscow Department of Health. To identify predictors of RCC, 100 patients undergoing hemodialysis treatment over the specified period were randomly selected as a control group. Results. Significant risk factors for RСС were only male (AOR 5.1; 95%CI 1.34-14.4; p=0.015) and acquired cystic kidney disease (ACKD), (AOR 3.2; 95% CI 1.56-16.43; p=0.008). There was a bimodal increase in the frequency of RСС: in the first year after the start of hemodialysis and 5 years later. After nephrectomy, only in one case there was bleeding due to systemic hypocoagulation. Hyperkalemia was observed in 5 (19.2%) patients immediately after laparoscopic nephrectomy. Based on the TNM stage, the UICC nomogram (University of California Los Angeles Integrated Staging System) and the Leibovich score, a favorable cancer prognosis was observed in 17 (65.4%) patients. The median follow - up for patients after nephrectomy was 20 months (Q1-Q3: 8.5- 45 months), the minimum period was 2 months, and the maximum period was 72 months. In no case was there any progression of RCC. Conclusion. Male patients with ACDK are at high risk of developing RCC. After laparoscopic nephrectomy shown to control the level of potassium the blood. The vast majority of patients after surgical treatment have a favorable oncological prognosis and may be considered for kidney transplantation without an additional follow-up period.

Determining the Frequency of Acquired Cystic Kidney Disease in End Stage Renal Disease Patients on Hemodialysis at Dialysis Centre of Tertiary Care Hospital.

ObjectivesTo determine the frequency of acquired cystic kidney disease (ACKD) among patients of end-stage renal disease.MethodsThis cross-sectional study was conducted at the University of Lahore Teaching Hospital after approval from the ethical review committee. About 150 patients with end-stage renal disease fulfilling the inclusion criteria and undergoing three hemodialysis sessions per week for six months were approached. The patients underwent ultrasonography by the same consultant radiologist and the presence of acquired polycystic kidney disease was noted in the proforma. Data was stratified for age, gender and duration of hemodialysis and the chi-square test was applied.ResultsThe mean age of the study participants was 47.31±9.44 years and males were majority in number with 92 (61.3%). The acquired cystic kidney disease was noted in 20 (13%) participants. There was significant difference noted in different age groups as six (6.5%) patients in the 18-40 age group and 14 (24%) patients in the 40-80 age group have acquired kidney disease (p-value=0.002). No important association between ACKD, age, and gender were found. None of these patients had evidence of renal cell carcinoma, extrarenal cysts, retroperitoneal or intrarenal hemorrhage.ConclusionThere was a significant correlation between acquired cystic kidney disease and the duration of hemodialysis, and the chances of the development of acquired cystic kidney disease rise progressively with increasing time on hemodialysis.

Acquired Cystic Kidney Disease-associated Renal Cell Carcinoma (ACKD-RCC) Harbor Recurrent Mutations in KMT2C and TSC2 Genes.

Individuals with acquired cystic kidney disease (ACKD) in the setting of end-stage renal disease (ESRD) have a high risk of developing renal cell carcinoma (RCC). ACKD-RCC is considered a distinct renal neoplasm in the International Society of Urologic Pathologists (ISUP)-World Health Organization (WHO) classification of kidney tumors which may behave aggressively. Since its original description, there have been multiple case reports and series published; however, the pathogenesis of this neoplasm is uncertain and there is limited data on the genetic aberrations of this tumor. Herein, we present our experience with ESRD kidneys, with emphasis on ACKD-RCC, associated cysts, and the somatic mutation analysis of a subset of ACKD-RCCs using next-generation sequencing. Our data on 59 cases with ESRD that underwent nephrectomy, shows that ACKD-RCC represents more than half of the tumors (25/46; 54%) developing in ESRD, followed by papillary RCC (13; 28%). History of dialysis, male sex, and African American race were potential risk factors for developing ACKD-RCCs. Further, ACKD-RCC-like cysts are possible precursors of RCCs in the ACKD setting noted in 40 of 46 (87%) cases with tumors. Next-generation sequencing analysis revealed recurrent mutations in the KMT2C gene in 4 of 5 ACKD-RCCs (80%), exclusively exhibiting cribriform "sieve-like" morphology; whereas the case negative for KMT2C mutations exhibited "type 2" papillary RCC morphology and lacked "sieve-like" growth pattern. Pathogenic mutations in TSC2 were the second common abnormality (3/5; 60%), often coexisting with KMT2C mutations. Deleterious mutations in additional genes such as CBL, PDGFRA, and SYNE1, etc. were noted but were nonrecurrent and always coexisted with mutations in KMT2C or TSC2. To conclude, our study highlights that mutations in a chromatin-modifying gene KMT2C may potentially be oncogenic drivers for the development of ACKD-RCC with classic sieve-like morphology. In addition, pathogenic mutations in TSC2 possibly play a role in the development of cysts/tumors in a subset of ACKD patients. If corroborated in larger cohorts, these findings would be useful in planning surveillance and early intervention in ESRD patients developing ACKD.

RENAL CELL CARCINOMA WITH ONCOCYTIC CELL TUMOR IN ASEMPTOMATIC DIALYSIS PATIENT

Kronik böbrek yetmezlikli ve renal replasman tedavisi gören hastalarda kardiyovasküler hastalıklar (%56), infeksiyonlar (%11.2) ve maligniteye (%8) bağlı artmış mortalite oranları söz konusudur. Diyaliz süresi uzadıkça malignite görülme oranı da artar. Diyaliz hastalarında renal hücreli karsinom (RCC) prevalansının genel popülasyona göre daha yüksek olduğu bilinmektedir. Edinilmiş kistik böbrek hastalığı (ACKD) sıklığı diyaliz süresi ile artar; CKD ile ilişkili RCC erkeklerde daha sık görülür; sıklıkla bilateral ve multisentriktir. Renal hücreli tümörlerden olan onkositom varyant bir tümör olarak kabul edilmesine rağmen histopatolojik olarak malign tümör özellikleri gösterebilir. Bu yazıda uzun süreden beri hemodiyaliz tedavisi gören ve yakınması olmayan hastada rastlantısal olarak saptanan renal kitleler anlatılmaktadır.

MP32-04 RENAL CELL CARCINOMA IN THE NATIVE KIDNEY IN DIALYSIS AND KIDNEY TRANSPLANT RECIPIENTS. COMPARISON OF PATHOLOGICAL FEATURES AND ACKD INFLUENCE.

You have accessJournal of UrologyTransplantation & Vascular Surgery: Renal Transplantation & Vascular Surgery II1 Apr 2016MP32-04 RENAL CELL CARCINOMA IN THE NATIVE KIDNEY IN DIALYSIS AND KIDNEY TRANSPLANT RECIPIENTS. COMPARISON OF PATHOLOGICAL FEATURES AND ACKD INFLUENCE. Kleiton Yamaçake, William Nahas, Ioannis Antonopoulos, Hideki Kanashiro, Raphael Kato, Renato Falci, and Affonso Piovesan Kleiton YamaçakeKleiton Yamaçake More articles by this author , William NahasWilliam Nahas More articles by this author , Ioannis AntonopoulosIoannis Antonopoulos More articles by this author , Hideki KanashiroHideki Kanashiro More articles by this author , Raphael KatoRaphael Kato More articles by this author , Renato FalciRenato Falci More articles by this author , and Affonso PiovesanAffonso Piovesan More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2016.02.1299AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Renal Cell Carcinoma (RCC) has a high incidence in patients with acquired cystic kidney disease (ACKD) due to hemodialysis. It has been reported that these tumors are less aggressive in RCC patients with normal renal function. We studied the clinical and pathologic outcomes of patients who developed RCC during dialysis and those who were diagnosed after transplantation. Groups were treated surgically for RCC in the native kidney METHODS Between January 1991 and December 2011, 2580 renal transplants were performed. Twenty-four patients underwent radical nephrectomy for a suspected renal mass in the native kidney. In 12 of these patients, the diagnosis and surgery were carried out prior to transplantation during the dialytic period (Group 1) and the remaining were carried out after transplantation (Group 2). In Group 2, 10 out of 12 patients with a suspected renal mass were confirmed to have RCC. One and 2 patients had bilateral disease in Group 1 and 2, respectively. This comprised 11 and 14 RCC in Groups 1 and 2, respectively. RESULTS Demographic data were similar in both groups. Mean period from transplant and radical nephrectomy in Group 1 was 97.6± 75.55(2-254) months. The mean duration of dialysis was higher in Group 1 (48.5± 15.7vs 26.22± 21.98, p= 0.147) and also had a higher association with ACKD (78.57% vs 18%, p= 0.0048). We observed a higher incidence of clear cell carcinoma in the dialytic group (92.7% vs 72.7%, p= 0.281). Papillary was present in 1 (Group 1) and 3 (Group 2) patients. Tumor multifocality occurred only in Group 1 (6 out of 14). The stage, Fuhrman grade, vascular invasion, necrosis, and size were similar in both groups. 13 RCC with ACKD were compared to 12 tumors without ACKD. There was no statistically difference regarding: age, gender, duration of dialisys, medical history, histology type, tumor size, stage, Fuhrman grade, vascular invasion and necrosis. Mean duration of dialysis was higher in patients with ACKD (48.2±44.09 vs 28.27±21.71 months, p= 0.3). Tumor multifocallity was observed only in patients with ACKD (p=0.0149). The mean follow up was 65.5± 42.31(8-156) months in Group 1 and 58± 42.51(2-145) months in Group 2. CONCLUSIONS Despite similar outcomes, it is clear that the renal masses that are acquired after renal transplantation have a different pathology than those acquired during dialysis. The low association with ACKD, low multiplicity and pathologic pattern are much more similar to normal kidney function tumors. © 2016FiguresReferencesRelatedDetails Volume 195Issue 4SApril 2016Page: e429 Advertisement Copyright & Permissions© 2016MetricsAuthor Information Kleiton Yamaçake More articles by this author William Nahas More articles by this author Ioannis Antonopoulos More articles by this author Hideki Kanashiro More articles by this author Raphael Kato More articles by this author Renato Falci More articles by this author Affonso Piovesan More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Renal cysts: Sonographic evaluation and classification in Sudanese adults

Background: Renal cysts were common findings in the kidneys encountered in daily routine ultrasound examination. The majority is benign, but they can become complicated in the case of infection, hemorrhage, and ischemia. Sonographic evaluation plays a great role in classification and characterization of these lesions. Objective: The aim was to classify the renal cysts using ultrasound, to analyze the frequency of symptoms and signs, and to identify the significant factors. Materials and Methods: This is a cross-sectional prospective study conducted from September 2014 to December 2015. A total of 105 patients (78 males and 27 females) have been examined with ultrasound and confirm diagnosed with renal cysts. The sonographic criteria used for assessing renal cysts were wall-definition, thickness of septa, acoustic enhancement, shape and numbers of cysts, in addition to the genetic history of the family. Results: The incidence of renal cysts is significantly higher in male than female (74% vs. 26%, P = 0.01). Renal cysts were common in patients above 50 years old (80%). Aging was a significant factor of the renal cyst ( P = 0.03) and size of the cysts was not significantly associated with age ( P = 0.261). The majority of cysts was solitary (66%), unilateral (86%), and located in the right kidney (49%). The incidence of cortical simple renal was 73%, para-pelvic simple cysts was 17%, autosomal-dominant polycystic kidney disease (ADPKD) was 4.76%, and acquired cystic kidney disease (ACKD) was 4.76%. The majority of simple renal cysts were asymptomatic (75%). Hypertension and flank pain were the common symptoms of ADPKD and ACKD. Conclusion: Age and gender were significant risk factors of renal cysts. ADPKD and ACKD were less frequent among Sudanese adults. Hypertension and flank pain were the most common symptoms and signs of ADPKD and ACKD. Ultrasound plays an effective role to classify and assess renal cysts that help in management and follow-up.

Letter to the editor: spontaneous renal haemorrhage in end-stage renal disease.

Letter to the editor: spontaneous renal haemorrhage in end-stage renal disease.

A Case of Egg-Shell Calcification of Renal Cell Carcinoma in Continuous Ambulatory Peritoneal Dialysis Patient with Sclerosing Encasulating Peritonitis

Acquired cystic kidney disease (ACKD), a common complication in patients with end-stage renal disease, is characterized by more than three kidney cysts and normal or decreased sizes of both kidneys without any familial history of cystic kidney disease. In autosomal dominant polycystic kidney disease (ADPKD), however, both kidneys are usually enlarged. Extrarenal manifestations are common in ADPKD, including hepatic cysts, seminal vesicle cysts, mitral valve prolapse. A 40-year-old man presented to the emergency clinic at Inha University Hospital with severe abdominal pain, nausea, and vomiting for 3 days. He had been undergoing continuous ambulatory peritoneal dialysis (CAPD) for 15 years, but it was recently changed to hemodialysis owing to sclerosing encapsulating peritonitis (SEP). Radiologic imaging studies revealed bilateral enlarged kidneys with multiple eggshell calcified cysts and some hepatic cysts, which suggested ADPKD. He underwent left nephrectomy, and pathological tests revealed ACKD-associated renal cell carcinoma (RCC) confined to the resected kidney. He was treated with steroids for SEP, and the symptoms resolved. We herein report a case of ACKD—resembling ADPKD—that progressed to RCC in a patient with concurrent SEP who had been undergoing CAPD for 15 years.

Haemangiomas in kidneys with end-stage renal disease: a novel clinicopathological association

The study of haemangiomas in end-stage renal disease (ESRD). Twenty ESRD nephrectomies from 16 patients (aged 9 months-68 years) were due to hypertension (four), focal segmental glomerulosclerosis (four), lupus nephritis (three), diabetes (one), IgA nephropathy (one), hereditary nephritis (one), congenital nephrotic syndrome (one) and unknown cause (one). Haemangiomas appeared as a single mass (15), two masses (one), three masses (one), four masses (two) and eight masses (one) per kidney. Tumours measured 0.2-3.5 cm. Four patients had bilateral haemangiomas. All tumours were in the medulla and often abutted renal sinus fat. All except one of the tumours were anastomosing haemangiomas, showing isolated or interconnected sinusoidal capillary-sized vascular channels lined by a single layer of benign cuboidal CD34(+) , CD31(+) , D2-40(-) endothelial cells, separated by loose stroma with spindle cells. One tumour was a cellular capillary haemangioma. Intravascular growth was seen in nine specimens. All haemangiomas had extramedullary haematopoiesis. Acquired cystic kidney disease (ACKD) was seen in 11 kidneys (nine patients), renal cell carcinoma (RCC) in five, ACKD-associated RCC precursors in three, Wilms' tumour in one and papillary adenomas in five. Anastomosing haemangioma appears as a distinctive clinicopathological entity developing in kidneys with ESRD, with or without ACKD.

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.

The Authors Reply: Renal Cell Carcinoma in Kidney Transplant Recipients and Dialysis Patients

To the editor: A limitation of our article is the absence of a control group and that this was not a prospective study. Our study was an insufficient randomized controlled cohort trial for acquired cystic kidney disease (ACKD). Our study would be better accepted if it was designed in a prospective manner, which would contain follow-up data for about 7,000 patients with end-stage renal disease (ESRD) for 14 years, with separate comparisons of the transplant group, dialysis-only group, and renal cancer patient group who underwent nephrectomy for ACKD. Because the study was performed in a retrospective manner, there was no strict protocol for imaging study. Furthermore, the diagnosis of ACKD in the native kidney was usually made by associated symptoms. This study made comparisons between the dialysis group and the transplanted group, who had undergone nephrectomy, retrospectively. Kojima et al. [1] observed that the incidence of renal cell carcinoma (RCC) in the native kidney was high in transplant recipients and reported that the incidence rate was high in dialysis patients. It was shown that 44 (1.68%) of 2,624 ESRD patients followed up during dialysis had developed RCC an average of 11.2 years after dialysis, and 36 of these cases (81.8%) were associated with ACKD. A positive correlation with the incidence rate of RCC was also found among transplant recipients. In 2007, Ianhez et al. [2] reported that 10 of 1375 patients developed RCC of the native kidney an average of 8.4 years after the renal replacement therapy, and all the RCC cases were associated with ACKD. In 1990, Matson and Cohen [3] investigated ACKD development and its correlation with RCC among ESRD patients. They demonstrated that ACKD was closely correlated with the duration of renal replacement therapy but not with the type of dialysis that was performed. Many studies on transplantation and ACKD have also reported that ACKD is observed more frequently, and the incidence rate of RCC is higher, in a kidney transplantation group than in a control group. Heinz-Peer et al. [4] analyzed 96 patients with ACKD detected by ultrasonography out of 385 renal transplant recipients in 1995 and found that the incidence of ACKD was higher with longer duration times of dialysis before kidney transplantation in older subjects and in males. The presence of ACKD was related to the occurrence of renal cancer, and the longer the duration of dialysis, the more likely the patient was to acquire ACKD. Only a few studies have been published regarding how renal transplantation itself affects ACKD, and yet, there is no definite answer as to the effects of renal transplantation on RCC. Our reports show that the frequency of ACKD was lower in the transplanted patient group than in the dialysis group, which still requires further explanation concerning the pathogenesis. Our institution follows the protocol for ESRD patients to undergo an annual ultrasonogram. This protocol is based on the results of this study and its main intent is to detect ACKD patients earlier. Therefore, we perform annual ultrasonograms for both dialysis patients and kidney transplantation patients. In the future, we hope that these approaches will contribute to the research and treatment in this field. REFERENCES

Letter to the Editor: Is Routine Screening Necessary for Renal Cell Carcinoma in End-Stage Renal Disease Patients?

To the editor: I read with great interest the original article by Lee et al. [1] published recently in your most valuable journal entitled Renal cell carcinoma in kidney transplant recipients and dialysis patients. It seems that the authors believe that routine screening is necessary for renal cell carcinoma (RCC) among patients with end-stage renal disease (ESRD). It is suggested that features such as increasing age, male gender, previous exposure to carcinogens, genetic predisposition, acquired cystic kidney disease (ACKD), and older age of the donor are common and important risk factors for RCC in kidney transplant patients [2,3]. The correlation of ACKD with the occurrence of RCC is stronger in dialysis patients than in kidney transplant recipients [1]. It is reported that the prevalence of RCC in the ESRD population is 3% to 4%, and that it is similar in dialyzed and transplanted patients, which corresponds to a rate 100 times that in the general population [4]. It is very interesting that clinical, pathological, and outcome features of RCCs arising in native kidneys of transplant patients seem to have many favorable properties when compared with those diagnosed in dialysis patients and patients from the general population. It is unclear whether this behavior is due to particular molecular pathways or to biases in relation to the mode of diagnosis, and this requires further research in the future [4,5]. When we focus on the prevalence and behaviors of RCC in ACKD patients, then we can understand why it is recommended in patients with ESRD that screening be performed only in patients with a long life expectancy and minimal major comorbidities with periodic ultrasound examination or computed tomography scans beginning during the third year on dialysis.