Tuberous Sclerosis Complex and the kidneys: what nephrologists need to know.

Tuberous sclerosis complex (TSC) is an autosomal dominant disease characterized by the development of hamartomas in the central nervous system, heart, skin, lungs, and kidneys and other manifestations including seizures, cortical tubers, radial migration lines, autism and cognitive disability. The disease is associated with pathogenic variants in the TSC1 or TSC2 genes, resulting in the hyperactivation of the mTOR pathway, a key regulator of cell growth and metabolism. Consequently, the hyperactivation of the mTOR pathway leads to abnormal tissue proliferation and the development of solid tumors. Kidney involvement in TSC is characterized by the development of cystic lesions, renal cell carcinoma and renal angiomyolipomas, which may progress and cause pain, bleeding, and loss of kidney function. Over the past years, there has been a notable shift in the therapeutic approach to TSC, particularly in addressing renal manifestations. mTOR inhibitors have emerged as the primary therapeutic option, whereas surgical interventions like nephrectomy and embolization being reserved primarily for complications unresponsive to clinical treatment, such as severe renal hemorrhage. This review focuses on the main clinical characteristics of TSC, the mechanisms underlying kidney involvement, the recent advances in therapy for kidney lesions, and the future perspectives.

Malignancy Diseases in Kidney Transplantation, Clinical Outcomes, Patient, and Allograft Survival: A Case-Control Study.

BACKGROUND: Malignancy is a well-known complication in patients after kidney transplantation (KT), but its effect on posttransplant outcomes, allograft, and patient survival remains unexplored. The aim of this study is to report the impact of the comorbidity on clinical outcome, function, and failure of an allograft kidney. METHODS: This case-control study included 101 KT patients. Twenty-six patients who developed cancer (CA) were assigned to the case group and 75 to the control group. Statistical analysis was performed using logistic regression models, and graft survival was analyzed using the Kaplan-Meier curve. RESULTS: Non-melanoma skin CA was the most common malignancy, accounting for almost 60% of cases, followed by stomach CA, prostate CA, and lymphoproliferative diseases (7.70% each). Difference in graft and patient survival was not significant between the two groups (P > .05). A tumor in nonfunctioning in the first nonfunctioning KT was identified in 1 KT patient with a second allograft and by anatomopathological was detect Fuhrman grade II renal cell carcinoma. This KT patient was in good clinical condition with serum creatinine level of 1.5 mg/dL. CONCLUSIONS: No association was observed between CA development and risk factors, including family history and smoking habit, and no differences in allograft and patient survival were found. Nevertheless, in our data, CA in KT patients occurred early after transplantation. Renal cell carcinoma in allograft failure was identified in a patient; that suggested that nephrectomy of kidney failure must be performed to avoid patient allosensitization and neoplasia. Thus, we suggest continuous screening of malignancy diseases for KT patients.

Circulating and Tumor-Infiltrating NK Cells From Clear Cell Renal Cell Carcinoma Patients Exhibit a Predominantly Inhibitory Phenotype Characterized by Overexpression of CD85j, CD45, CD48 and PD-1.

Although natural killer (NK) cells infiltrate clear cell renal cell carcinomas (ccRCC), the most frequent malignancy of the kidney, tumor progression suggests that they become dysfunctional. As ccRCC-driven subversion of NK cell effector functions is usually accompanied by phenotypic changes, analysis of such alterations might lead to the identification of novel biomarkers and/or targets in immuno-oncology. Consequently, we performed a phenotypic analysis of peripheral blood NK cells (PBNK) and tumor-infiltrating NK cells (TINK) from ccRCC patients. Compared to HD, PBNK from ccRCC patients exhibited features of activated cells as shown by CD25, CD69 and CD62L expression. They also displayed increased expression of DNAM-1, CD48, CD45, MHC-I, reduced expression of NKG2D, and higher frequencies of CD85j+ and PD-1+ cells. In addition, compared to PBNK from ccRCC patients, TINK exhibited higher expression of activation markers, tissue residency features and decreased expression of the activating receptors DNAM-1, NKp30, NKp46, NKp80 and CD16, suggesting a more inhibitory phenotype. Analysis of The Cancer Genome Atlas (TCGA) revealed that CD48, CD45, CD85j and PD-1 are significantly overexpressed in ccRCC and that their expression is associated with an NK cell infiltration signature. Calculation of z-scores revealed that their expression on PBNK, alone or combined, distinguished ccRCC patients from HD. Therefore, these molecules emerge as novel potential biomarkers and our results suggest that they might constitute possible targets for immunotherapy in ccRCC patients.

Immunoglobulin G4-related disease with recurrent uveitis and kidney tumor mimicking childhood polyarteritis nodosa: a rare case report.

INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is an condition rarely reported in children. Additionally, IgG4-RD may rarely mimic vasculitis in adults and may infrequently present with uveitis. In our service, 6,198 patients were followed-up and only one (0.0001%) of them had IgG4-RD. To our knowledge, the present IgG4-RD case was the first mimicking childhood polyarteritis nodosa (c-PAN) with recurrent uveitis and kidney tumor. CASE REPORT: We describe herein a 7-year-old boy that presented intermittent fever. He developed arthralgia, weight loss, myalgia, skin lesions and recurrent uveitis. Skin biopsy revealed necrotizing vasculitis in medium/small sized vessels associated with septal panniculitis suggesting cPAN. Prednisone and azathioprine were administered with improvement. At 11 years, he had persistent fever and abdominal angiotomography revealed a large tumor in left kidney and he was then submitted to nephrectomy. The renal histopathology showed lymphoplasmacytic and histiocytic proliferation with extensive areas of fibrosis, and lymphomonocitic phlebitis with presence of IgG4 in 43 plasmocyte cells, suggesting IgG4-RD. DISCUSSION: we present herein a unique case of a male pediatric patient with IgG4-RD with rare ocular, cutaneous and renal manifestations.

Giant Renal Angiomyolipoma Following Ovarian Stimulation Therapy.

Renal angiomyolipomas (AMLs) are benign tumors with higher prevalence in women. Female hormones have been shown to induce AML enlargement. This case refers to a 40-year-old woman with 4 left kidney AMLs, the larger ones with 1.0 and 1.3 cm. Ten months after ovarian stimulation for egg harvesting, a computed tomography revealed an 18-cm AML with large-caliber vessels. Given her high risk of AML bleeding, the patient was submitted to selective arterial embolization, which turned out unsuccessful, supporting a plan of nephron-sparing surgery. Our case highlights the pro-growth effects of female hormones on AML, with particular emphasis to ovarian stimulation.

Obesity and kidney disease: hidden consequences of the epidemic.

Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease and also for Chronic kidney disease. A high body mass index is one of the strongest risk factors for new-onset Chronic kidney disease. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing Chronic kidney disease in the long-term. The incidence of obesity-related glomerulopathy has increased ten-fold in recent years. Obesity has also been shown to be a risk factor for nephrolithiasis, and for a number of malignancies including kidney cancer. This year the World Kidney Day promotes education on the harmful consequences of obesity and its association with kidney disease, advocating healthy lifestyle and health policy measures that makes preventive behaviors an affordable option.

Effects of malnutrition on treatment-related morbidity and survival of children with cancer in Nicaragua.

BACKGROUND: Most children with cancer live in resource-limited countries where malnutrition is often prevalent. We identified the relationship between malnutrition and treatment-related morbidity (TRM), abandonment of therapy, and survival of children with cancer in Nicaragua to better inform targeted nutritional interventions. PROCEDURE: We conducted a retrospective review of patients aged 6 months to 18 years with newly diagnosed acute lymphoblastic leukemia, acute myeloid leukemia (AML), Wilms tumor, Hodgkin lymphoma, or Burkitt lymphoma (BL) who were treated between January 1, 2004, and December 31, 2007 at Children's Hospital Manuel de Jesus Rivera in Managua, Nicaragua. Statistical analysis examined the relations among nutritional status and cancer type, risk category, TRM, and event-free survival (EFS). RESULTS: Sixty-seven percent of patients (189/282) were malnourished at diagnosis. Malnutrition was highest among patients with Wilms tumor (85.7%), BL (75%), and AML (74.3%). A total of 92.2% of patients (225/244) experienced morbidity during the first 90 days. Malnutrition was associated with severe infection (P = 0.033). Severely malnourished patients had ≥grade 3 TRM on more days (P = 0.023) and were more likely to experience severe TRM on >50% of days (P = 0.032; OR, 3.27 [95% CI, 1.05-10.16]). Malnourished patients had inferior median EFS (2.25 vs. 5.58 years; P = 0.049), and abandoned therapy more frequently (P = 0.015). CONCLUSIONS: In Nicaragua, pediatric oncology patients with malnutrition at diagnosis experienced increased TRM, abandoned therapy more frequently, and had inferior EFS. Standardized nutritional evaluation of patients with newly diagnosed cancer and targeted provision of nutritional support are essential to decrease TRM and improve outcomes.

Obesity and kidney disease: hidden consequences of the epidemic / Obesidad y enfermedad renal: las consecuencias ocultas de una epidemia

Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease and also for Chronic kidney disease. A high body mass index is one of the strongest risk factors for new-onset Chronic kidney disease. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing Chronic kidney disease in the long-term. The incidence of obesity-related glomerulopathy has increased ten-fold in recent years. Obesity has also been shown to be a risk factor for nephrolithiasis, and for a number of malignancies including kidney cancer. This year the World Kidney Day promotes education on the harmful consequences of obesity and its association with kidney disease, advocating healthy lifestyle and health policy measures that makes preventive behaviors an affordable option.

La obesidad se ha convertido en una epidemia mundial, y se ha proyectado que su prevalencia se incrementrá en 40% en la próxima década. Esta creciente prevalencia supone implicaciones tanto para el riesgo de desarrollo de diabetes mellitus y enfermedades cardiovasculares como para el desarrollo de Enfermedad renal crónica. Un elevado índice de masa corporal es uno de los factores de riesgo más importantes para el desarrollo de enfermedad renal crónica. En individuos afectados por la obesidad, tiene lugar una hiperfiltración compensatoria necesaria para alcanzar la alta demanda metabólica secundaria al aumento del peso corporal. El incremento de la presión intraglomerular puede generar daño renal y elevar el riesgo de desarrollar enfermedad renal crónica a largo plazo. La incidencia de glomerulopatía asociada a obesidad se ha incrementado 10 veces en los últimos años. Asimismo se ha demostrado que la obesidad es un factor de riesgo para el desarrollo de nefrolitiasis y un número de neoplasias, incluyendo cáncer renal. Este año, el Día Mundial del Riñón promueve la educación acerca de las consecuencias nocivas de la obesidad y su asociación con la enfermedad renal, abogando por un estilo de vida saludable y la implementación de políticas públicas de salud que promuevan medidas preventivas alcanzables.

Aumento renal bilateral como manifestação de leucemia linfocítica aguda / Bilateral renal increase as a manifestation of acute lymphocytic leukemia

As leucemias são o tipo mais frequente de câncer em crianças e adolescentes. A leucemia linfocítica aguda é a forma mais comum de leucemia na infância. A doença surge normalmente entre dois e quatro anos de idade, sendo incomum antes de um ano de vida. As manifestações mais comuns incluem febre, fadiga, letargia, dor óssea e articular. Em 50% dos casos, há hepato e/ou esplenomegalia e linfonodomegalias. Este relato de caso fala sobre uma menina de nove meses que foi levada à emergência do hospital por distensão abdominal, fraqueza em membros inferiores e constipação duas semanas antes. Apresentava massas abdominais endurecidas em flancos, abaulamento em região fronto-temporal direita e linfonodomegalias cervicais. A primeira impressão foi de nefroblastoma bilateral (Tumor de Wilms). A ecografia abdominal e a tTC de abdome mostraram aumento de volume renal bilateral e descartaram nefroblastoma. Em setor de oncologia pediátrica, foi realizada imunofenotipagem compatível com leucemia/linfoma linfoblástico B. No líquor havia 400 células p/uL com 81% de blastos. A avaliação neurológica e a RNM de crânio e neuro-eixo descartaram alteração que justificasse a paresia de membros inferiores. No décimo dia de tratamento, os rins já haviam reduzido até o limite superior da normalidade. O abaulamento da face já havia desaparecido. A paciente apresentou anemia, neutropenia, plaquetopenia e hipoalbuminemia severas. Ao final da indução, apresentou sepse fúngica e bacteriana evoluindo para choque séptico e parada cardiorrespiratória não responsiva às manobras de reanimação. Não foi realizado medulograma no final da indução por piora do quadro clínico e posterior óbito (AU)

Leukemias are the most frequent type of cancer in children and adolescents. Acute lymphocytic leukemia is the most common form of childhood leukemia. The disease usually arises between two and four years of age, being uncommon before one year of life. The most common manifestations include fever, fatigue, lethargy, bone and joint pain. In 50% of cases, there is hepatomegaly and/or splenomegaly and lymph node enlargement. This case report tells of a nine-month old girl who was taken to the hospital emergency room due to abdominal distension, weakness in the lower limbs, and constipation for two weeks. She had abdominal masses that were hardened on the flanks, bulging in the right fronto-temporal region and cervical lymph node enlargements. The first impression was bilateral nephroblastoma (Wilms tumor). Abdominal ultrasound and abdominal CT showed bilateral renal volume increase and ruled out nephroblastoma. In the pediatric oncology sector, immunophenotyping compatible with lymphoblastic leukemia/lymphoma was performed. In the CSF there were 400 cells/L with 81% blasts. Neurological evaluation and MRI of the skull and neuro-axis ruled out alterations justifying lower limb paresis. By the tenth day of treatment, the kidneys had already reduced to the upper limit of normal. The bulging of the face was gone. The patient presented severe anemia, neutropenia, thrombocytopenia and hypoalbuminemia. At the end of the induction, she presented fungal and bacterial sepsis evolving to septic shock and cardiorespiratory arrest unresponsive to resuscitation maneuvers. No myelogram was performed at the end of the induction due to worsening of the clinical picture and subsequent death (AU)

Clinical study of 20 patients with incontinentia pigmenti.

BACKGROUND: Incontinentia pigmenti (IP) is a rare genodermatosis with early prenatal lethality in affected males. Clinical manifestations are usually more exuberant in sporadic than in familial cases. Cutaneous manifestations occur in all sporadic cases and about 96% of familial cases. As well as the skin, other tissues arising from the neuroectoderm may be affected. OBJECTIVES: This study was designed to evaluate dermatologic, dental, neurologic, and ophthalmologic manifestations in patients with IP. METHODS: Findings in IP patients and family members also diagnosed with IP in Porto Alegre, Brazil, during 2003-2012, were analyzed. RESULTS: Thirteen children and seven relatives were diagnosed with IP; 38.4% of cases were familial, and 61.5% were sporadic. Mean ± standard deviation follow-up was 46.08 ± 39.47 months. Frequencies of 100% and 85.7% for dermatologic manifestations, 23.0% and 0% for neurologic manifestations, 62.5% and 71.4% for dental manifestations, and 11.1% and 42.8% for ophthalmologic manifestations were found in affected children and relatives, respectively. Associated diseases include Wilms' tumor, myasthenia gravis, Still's syndrome, and congenital hypothyroidism. CONCLUSIONS: These findings reinforce the heterogeneity of dermatologic findings and the numerous extracutaneous manifestations requiring a multidisciplinary approach. The follow-up of patients with IP is important in the detection of serious associated diseases. The relationships between these disorders and IP raise the need for additional longitudinal studies with longterm monitoring of these patients. The management of IP in clinical practice may benefit from early efforts to detect associated diseases.

Tuberous Sclerosis Complex: State-of-the-Art Review with a Focus on Pulmonary Involvement.

Tuberous sclerosis complex (TSC) is an autosomal-dominant neurocutaneous disease with high phenotypic variability. The incidence is approximately one in 5000-10,000 births. TSC is characterized by widespread hamartomas and benign or rarely malignant neoplasms affecting various organs, most commonly the brain, skin, retinas, kidneys, heart, and lungs. The wide range of organs affected reflects the roles of TSC1 and TSC2 genes in the regulation of cell proliferation and differentiation. Clinical diagnostic criteria are important because genetic testing does not identify the mutation in up to 25% of patients. Imaging is pivotal, as it allows a presumptive diagnosis of TSC and definition of the extent of the disease. Common manifestations of TSC include cortical tubers, subependymal nodules, white matter abnormalities, retinal abnormalities, cardiac rhabdomyoma, lymphangioleiomyomatosis (LAM), renal angiomyolipoma, and skin lesions. Pulmonary involvement consists of LAM and, less commonly, multifocal micronodular pneumocyte hyperplasia (MMPH), which causes cystic and nodular diseases, respectively. Recent reports indicate that pulmonary LAM is found by computed tomography in up to 35% of the female patients with TSC. MMPH is rare and may be associated with LAM or, less frequently, occurs as an isolated pulmonary manifestation in women with TSC. Dyspnea and pneumothorax are common clinical presentations of LAM, whereas MMPH is usually asymptomatic. The aim of this review is to describe the main clinical, imaging, and pathological aspects of TSC, with a focus on pulmonary involvement.

Association of TP53 polymorphisms on the risk of Wilms tumor.

BACKGROUND: Molecular factors influencing Wilms tumor (WT) development remain largely unknown. TP53 mutations seem to be restricted to the anaplastic WT subtype. However, TP53 polymorphisms do not have a defined role in the disease. PROCEDURE: To assess the impact of TP53 mutations and polymorphisms (PIN2, PIN3, and PEX4) on risk of development, age at diagnosis, and survival in WT, we analyzed 46 blood DNA samples and 31 fresh tumor DNA samples from 52 patients with WT. Sequencing of TP53 exons 2-11 was performed. RESULTS: Tumor DNA analysis revealed TP53 pathogenic missense mutations (p.V197M, p.R213Q, p.R248W, and p.R337C) in four samples (12.9%). Blood DNA samples revealed a novel intronic mutation, IVS2 + 37C > T, in one patient (2.2%). Bilaterality was associated with a twofold decrease in survival (P = 0.00037). Diffuse anaplasia also presented a lower survival probability compared to patients with non-anaplastic tumors, or with focal anaplasia (P = 0.045). Patients with a TP53 somatic mutation showed survival probability of 37.5% versus 85.0% for patients with no somatic mutations, although the difference was not statistically significant (P = 0.0706). PIN3 duplicated allele was associated with a 20-month later mean age at diagnosis (P = 0.0084). TP53 PEX4 C allele showed an increased risk for WT development (P = 0.0379). No relationship was found between survival and gender, age at diagnosis, or the less frequent alleles of PIN2, PIN3, and PEX4. CONCLUSIONS: Our results demonstrate an association between PIN3 and age at diagnosis, as well as an association of PEX4 and risk of development of WT.

Molecular basis for the treatment of renal cell carcinoma.

Renal cell carcinoma (RCC) is a heterogeneous malignancy whose incidence rate has notably increased in recent years without any evident reason. Traditionally, RCC has been resistant to classic treatments (chemotherapy, radiotherapy and hormonal therapy), with only a small percentage of patients benefiting from cytokine therapy. Different hereditary syndromes have been associated with RCC, Von Hippel Lindau (VHL) being the most important syndrome. Understanding key molecular pathways implicated in the tumorigenesis of RCC has crystallised in the development of more effective therapies. Specifically, drugs targeting VEGF (bevacizumab, sunitinib, sorafenib, axitinib, pazopanib) and PI3K-mTOR (temsirolimus and everolimus) have become the cornerstone of renal cancer treatment.

Urothelial carcinoma transmission via kidney transplantation.

Transmission of urothelial carcinoma via solid organ transplant has never been reported in the literature to our knowledge. We report a case of transmission of this tumour to a kidney recipient. The donor was a 37-year-old woman, victim of a subarachnoid haemorrhage. The recipient was a 21-year-old girl, with a history of chronic kidney disease secondary to neurogenic bladder. This fatality has been rarely described in literature, but never with this histological type of cancer. Nowadays, with the expanded criteria for donation, older people are accepted as donor because of the shortage of organs. However, this may increase the likelihood of the number of cancer transmission.

Urological conservative management of a patient with tuberous sclerosis complex (Bourneville disease).

OBJECTIVE: To report the clinical and radiological data of a 23-year-woman with bilateral angiomyolipoma and Tuberous Sclerosis with conservative treatment. METHODS: The patient underwent conservative urological management, based on clinical and complementary tests data (ultrasonography, CT scanning, excretory urogram, and laboratory). Monitoring was carried out in external consultation by the urooncology group. In some occasions she was hospitalized due to distant geographical factors. RESULTS: This patient provides long-term outcome of AML with Tuberous Sclerosis followed by repeated evaluation to define the conservative management or not. Ultrasound and CT scan were two basic studies to maintain conservative treatment, as well as a good clinical evaluation. CONCLUSIONS: Renal lesions associated with tuberous sclerosis are frequent and serious. Monitoring should be based on a careful monitoring since the renal lesions are the second death cause after the lesions of the nervous system. This case report provides long-term outcome of AML with tuberous sclerosis, that can be followed expectantly with repeated evaluations to define the clinical significance.

Tumor renal maligno: análise das características clínicas, anatomatológicas e sobrevida dos pacientes nefrectomizados / Malignant renal tumor: analyze of clinical characteristics, anatomopathological and survival of nephrectomized patients

Analisar as características dos pacientes nefrectomizados por tumor renal maligno, para avaliar possibilidade de implantação do nomograma desenvolvido pelo Hospital Memorial Sloan-Kettering Cancer Center. Análise dos pacientes nefrectomizados por tumor pelos grupos de Urologia e Oncologia do Hospital Universitário de Taubabé e Hospital Regional do Vale do Paraíba, no período de 1998 a 2003, por análise de prontuário e contato telefônico. Na comparação de percentagens entre o estado atual dos pacientes (83,33 por cento) e a média da sobrevida estimada pelo nomograma (77,18 por cento) não houve significância estatística (p = 1). O nomograma pode ser aplicado para esta população.

In order to analyze the characteristics of the patients with malignant renal tumor submitted the nephrectomy surgery, and evaluate the possibility implantation of the nomogram developed by the Memorial Sloan-Kettering Cancer Center. The analysis of patients who were submitted to surgery for tumor by the group of Urology in Taubaté University Hospital and Vale do Paraíba's Regional Hospital from 1998 to 2003, for handbooks and telephonic contact. There was not significant diference (p = 1) between the actual condition of the patients (83.33 per cent) and the average of survivors estimated by the nomogram (77.18 per cent). The nomogram is applied for this population of this study.

Renal medullary carcinoma: report of seven cases from Brazil.

We report seven cases of renal medullary carcinoma collected from several institutions in Brazil. In spite of a relatively high incidence of sickle cell trait in Brazil, this is a rare tumor. All patients were males between the ages of 8 and 69 years (mean 22 years). From the collected information, the most frequent presenting symptoms were gross hematuria and flank or abdominal pain. The duration of symptoms ranged from 1 week to 5 months. Most of the tumors were poorly circumscribed arising centrally in the renal medulla. Size ranged from 4 to 12 cm (mean 7 cm) and hemorrhage and necrosis were common findings. All seven cases described showed sickled red blood cells in the tissue and six patients were confirmed to have sickle cell trait. All cases disclosed the characteristic reticular pattern consisting of tumor cell aggregates forming spaces of varied size, reminiscent of yolk sac testicular tumors of reticular type. Other findings included microcystic, tubular, trabecular, solid and adenoid-cystic patterns, rhabdoid-like cells and stromal desmoplasia. A peculiar feature was suppurative necrosis typically resembling microabscesses within epithelial aggregates. The medullary carcinoma of the 69-year-old patient was associated with a conventional clear cell carcinoma. To our knowledge, this association has not been previously reported and the patient is the oldest in the literature. The survival after diagnosis or admission ranged from 4 days to 9 months. The 8-year-old African-Brazilian patient with a circumscribed mass is alive and free of recurrence 8 years after diagnosis. This case raises the question whether a periodic search for renal medullary carcinoma in young patients who have known abnormalities of the hemoglobin gene and hematuria could result in an early diagnosis and a better survival.

Acquired cystic renal disease complicated with renal cell carcinoma.

Renal cell carcinoma is the most feared complication in patients with acquired renal cystic disease. We present a 73-year-old man with end stage renal disease in chronic hemodialysis for eleven years that was admitted to the hospital because of hematuria and right flank pain. Two months before admission the abdominal CT reported atrophic changes of the right kidney with multiple masses. A right laparoscopic nephrectomy demonstrated renal cystic disease associated with multifocal cystic renal cell carcinoma, clear cell, nuclear grade II. There was no extra renal extension or renal pelvis involvement. Patients with end-stage renal disease who have been on hemodialysis for three to five years should undergo yearly screening with ultrasonography. Once the ultrasonogram becomes positive for cysts, the more sensitive contrast enhanced CT scanning should be performed yearly to screen for the possible occurrence of renal cell carcinoma.