Wilms' Tumour
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Wilms' tumour is a cancer of the kidney which is very different to adult kidney cancer. Most patients are under 5 years of age at diagnosis, though Wilms' tumour is sometimes seen in older children and occasionally in young adults. In most cases only one kidney has disease (unilateral-Wilms' Tumour); but in some cases both kidneys are affected (bilateral-Wilms' tumour). A small minority of cases are known to be hereditary. Other less common kidney cancers in children include malignant rhabdoid tumours and clear cell sarcoma. Treatment for these is usually similar to that for Wilms' tumour.

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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Interiano RB, McCarville MB, Santos ND, et al.
Comprehensive renal function evaluation in patients treated for synchronous bilateral Wilms tumor.
J Pediatr Surg. 2017; 52(1):98-103 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
OBJECTIVES: The purpose of this study was to perform a comprehensive assessment of long-term renal function in patients treated at our institution for synchronous bilateral Wilms tumor (BWT) and to determine the optimal method for estimating glomerular filtration rate (eGFR).
METHODS: Surgical approach, adjuvant therapy, and pathology reports were reviewed for patients with at least six months follow-up from definitive surgery. eGFRs, as assessed by the Schwartz and Chronic Kidney Disease in Children (CKiD) formulas, were compared to measured GFR (mGFR) determined by (99m)Tc-DTPA scanning. Urine studies, including microalbumin, β-microglobulin, and FENa were also reviewed.
RESULTS: Forty-two patients were identified. Of 36 living patients, 28 (77.8%) had greater than 6months follow-up, with a median overall follow-up of 5.2years (range: 1.4-13.4). The median mGFR was 97mL/min/1.73m(2), while the median eGFRSchwartz and eGFRCKiD were 103.3mL/min/1.73m(2) and 79.7mL/min/1.73m(2), respectively, (p=0.13 and p=0.75, compared to mGFR). Eleven (39.3%) patients had at least one abnormal urine study (microalbumin >30μg/g creatinine, n=3; β-2 microglobulin >133μg/g creatinine, n=9; FENa>1%, n=4).
CONCLUSIONS: In our series, few patients had an abnormally low GFR. Neither method for estimating GFR gave a significantly different result from measured GFR, suggesting that the Schwartz equation is adequate, although specific urine tests may be more sensitive for detecting subtle renal dysfunction.
LEVEL OF EVIDENCE: Level IV - retrospective case series with no comparison group.

Related: Kidney Cancer

Richards MK, Goldin AB, Savinkina A, et al.
The association between nephroblastoma-specific outcomes and high versus low volume treatment centers.
J Pediatr Surg. 2017; 52(1):104-108 [PubMed] Related Publications
BACKGROUND: Though the volume-outcome relationship has been well-established in adults, low mortality rates and small sample sizes have precluded definitive demonstration in children. This study compares treatment-specific factors for children with nephroblastoma at high (HVC) versus low volume centers (LVC).
METHODS: We performed a retrospective cohort study comparing patients ≤18years with unilateral nephroblastoma treated at HVCs and LVCs using the National Cancer Data Base (1998-2012). Definitions of HVCs included performing above the median, the upper two quartiles, and the highest decile of nephroblastoma resections. Outcomes included nodal sampling, margin status, time to chemotherapy and radiation, and survival. Statistical analyses included χ(2), t-tests, generalized linear, and Cox regression models (p<0.05).
RESULTS: Of 2911 patients from 210 centers, 1443 (49.6%) were treated at HVCs. There was no difference in frequency of preoperative biopsy or days to radiation (p>0.05). High volume centers were more likely to perform nodal sampling (RR 1.04, 95%CI 1.01-1.08) and had fewer days to chemotherapy (RR 0.80, 95%CI 0.69-0.93). Five-year survival was similar (HVC: 0.93, 95%CI 0.92-0.94; LVC: 0.93, 95%CI 0.91-0.94).
CONCLUSIONS: HVCs were more likely to perform nodal sampling and had fewer days to chemotherapy. There was no difference in days to radiation or survival between centers.
LEVEL OF EVIDENCE: Level II (retrospective prognosis study).

Related: Kidney Cancer USA

Micale MA, Embrey B, Macknis JK, et al.
Constitutional 560.49 kb chromosome 2p24.3 duplication including the MYCN gene identified by SNP chromosome microarray analysis in a child with multiple congenital anomalies and bilateral Wilms tumor.
Eur J Med Genet. 2016; 59(12):618-623 [PubMed] Related Publications
Fewer than 100 patients with partial chromosome 2p trisomy have been reported. Clinical features are variable and depend on the size of the duplicated segment, but generally include psychomotor delay, facial anomalies, congenital heart defect, and other abnormalities. We report a 560.49 kb duplication of chromosome 2p in a 13 month-old male with hydrocephaly, ventricular septal defect, partial agenesis of the corpus callosum, and bilateral Wilms tumor. After discovery of bilateral renal masses at four months of age, the child underwent neoadjuvant chemotherapy followed by right radical nephrectomy that revealed triphasic Wilms' tumor. A needle core biopsy on one of two lesions on the left kidney also revealed Wilms tumor. A partial left nephrectomy revealed focally positive margins that necessitated left flank radiotherapy. The tumor karyotype was 46,XY,t(7;8)(q36;p11)[8]/46,XY [12] while his constitutional karyotype was 46,XY, suggesting that the t(7;8)(q36;p11) was associated with the malignancy. Single nucleotide polymorphism (SNP) chromosome microarray analysis of peripheral blood identified a maternally-inherited 560.49 kb chromosome 2p24.3 duplication that involved four OMIM genes: NBAS, DDX1, MYCNOS, and MYCN. SNP array analysis of the tumor revealed the same 2p24.3 duplication. At present, the now 5-year-old boy continues to do well without clinical or radiographic evidence of recurrent disease. This case is instructive because the child's health insurer initially denied authorization for chromosome microarray analysis (CMA), and it took more than one year before such authorization was finally granted. That initial decision to deny coverage could have had untoward health implications for this child, as the identification of constitutional MYCN duplication necessitated surveillance imaging for a number of pediatric malignancies associated with MYCN overexpression/dysregulation.

Related: Chromosome 2 Kidney Cancer MYCN (n-myc)

Iumanne S, Shoo A, Akoko L, Scanlan P
Case report: Xanthogranulomutous pyelonephritis presenting as "Wilms' tumor".
BMC Urol. 2016; 16(1):36 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is a rare renal tumor that arises as a complication of chronic obstructive pyelonephritis of uncertain etiology. It is primarily an adult tumor seen occasionally in children associated with urinary tract obstruction due to congenital urological anomalies, nephrolithiasis, and recurrent urinary tract infections. Radiologically, it may show neoplastic features such as those seen in common pediatric renal malignancies like wilms' tumor and renal cell carcinoma. This overlap in radiological manifestation frequently leads to misdiagnosis and delay in appropriate intervention. We report a case of a 3 years old boy who presented with history of recurrent urinary tract infections and a left renal mass initially thought to be Wilms' tumor.
CASE PRESENTATION: We present a case of a 3 years old boy admitted to the Pediatric oncology unit at Muhimbli National Hospital in Dar es Salaam, Tanzania with one year history of recurrent fever and urinary tract infection signs and symptoms refractory to antibiotic therapy. He was eventually found to have a left kidney mass detected at the District hospital by abdominal ultrasound performed to evaluate a flank mass that was felt by his mother. He was then referred to our unit for a suspicion of Wilms' tumor which finally turned out  to be a left kidney Xanthogranulomatous pyelonephritis. He underwent a successful left nephrectomy and was discharged from hospital in a stable clinical condition and remains asymptomatic at the time of submission of this case report.
CONCLUSION: This case report underscores the need for clinicians attending a febrile child with a renal mass that can be confused with common pediatric renal malignancies such as Wilms' tumor to broaden their differential diagnosis. The case also underlines the significance of individualized patient evaluation because this patient would have otherwise received preoperative chemotherapy under the International Society of Pediatric Oncology (SIOP) guidelines if the diagnosis of Wilms tumor was not ruled out.

Related: Kidney Cancer

Cresswell GD, Apps JR, Chagtai T, et al.
Intra-Tumor Genetic Heterogeneity in Wilms Tumor: Clonal Evolution and Clinical Implications.
EBioMedicine. 2016; 9:120-9 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
The evolution of pediatric solid tumors is poorly understood. There is conflicting evidence of intra-tumor genetic homogeneity vs. heterogeneity (ITGH) in a small number of studies in pediatric solid tumors. A number of copy number aberrations (CNA) are proposed as prognostic biomarkers to stratify patients, for example 1q+ in Wilms tumor (WT); current clinical trials use only one sample per tumor to profile this genetic biomarker. We multisampled 20 WT cases and assessed genome-wide allele-specific CNA and loss of heterozygosity, and inferred tumor evolution, using Illumina CytoSNP12v2.1 arrays, a custom analysis pipeline, and the MEDICC algorithm. We found remarkable diversity of ITGH and evolutionary trajectories in WT. 1q+ is heterogeneous in the majority of tumors with this change, with variable evolutionary timing. We estimate that at least three samples per tumor are needed to detect >95% of cases with 1q+. In contrast, somatic 11p15 LOH is uniformly an early event in WT development. We find evidence of two separate tumor origins in unilateral disease with divergent histology, and in bilateral WT. We also show subclonal changes related to differential response to chemotherapy. Rational trial design to include biomarkers in risk stratification requires tumor multisampling and reliable delineation of ITGH and tumor evolution.

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Teng WJ, Zhou C, Liu LJ, et al.
Construction of a protein-protein interaction network of Wilms' tumor and pathway prediction of molecular complexes.
Genet Mol Res. 2016; 15(2) [PubMed] Related Publications
Wilms' tumor (WT), or nephroblastoma, is the most common malignant renal cancer that affects the pediatric population. Great progress has been achieved in the treatment of WT, but it cannot be cured at present. Nonetheless, a protein-protein interaction network of WT should provide some new ideas and methods. The purpose of this study was to analyze the protein-protein interaction network of WT. We screened the confirmed disease-related genes using the Online Mendelian Inheritance in Man database, created a protein-protein interaction network based on biological function in the Cytoscape software, and detected molecular complexes and relevant pathways that may be included in the network. The results showed that the protein-protein interaction network of WT contains 654 nodes, 1544 edges, and 5 molecular complexes. Among them, complex 1 is predicted to be related to the Jak-STAT signaling pathway, regulation of hematopoiesis by cytokines, cytokine-cytokine receptor interaction, cytokine and inflammatory responses, and hematopoietic cell lineage pathways. Molecular complex 4 shows a correlation of WT with colorectal cancer and the ErbB signaling pathway. The proposed method can provide the bioinformatic foundation for further elucidation of the mechanisms of WT development.

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Nakayama DK, Bonasso PC
The History of Multimodal Treatment of Wilms' Tumor.
Am Surg. 2016; 82(6):487-92 [PubMed] Related Publications
Multimodal therapy-surgery, radiation therapy, and chemotherapy-the foundation of modern cancer treatment, has led to dramatic improvements in survival. How the three disciplines coalesced to conquer Wilms' tumor is a compelling story that includes two of history's greatest discoveries, X-rays and antibiotics. By the mid-20th century both fields had matured to where dedicated clinicians and creative scientists could apply them to Wilms' tumor and achieve successive improvements in survival. William Ladd was able to achieve a zero operative mortality by 1940, but was left with a 32 per cent survival with surgery alone. Robert Gross and Edwin Neuhauser combined surgery and radiotherapy and achieve 47 per cent survival rate in 1950. Sidney Farber and his colleagues added an antibiotic, dactinomycin, to the treatment regimen and reached 80 per cent survival rate in 1966. The National Wilms' Tumor Study, organized in 1968, was a multidisciplinary effort of surgeons, radiotherapists, and pediatric oncologists across the country. By the 1990s, the National Wilms' Tumor Study achieved survival rates above 95 per cent while minimizing long-term effects through shortening courses of chemotherapy and radiation. The story of Wilms' tumor serves as a paragon for all types of cancer, in both children and adults.

Related: Kidney Cancer

Chen SH, Hung IJ, Yang CP, et al.
Clinical features and long-term outcomes of bilateral Wilms tumor treated with Taiwan Pediatric Oncology Group protocols: A single center report.
Asia Pac J Clin Oncol. 2016; 12(3):300-7 [PubMed] Related Publications
AIMS: Wilms tumor (WT) is rare in Asia. Treatment of bilateral WT is challenging, and the treatment outcome of bilateral WT is rarely reported in low incidence areas.
METHODS: We enrolled patients with bilateral WT registered in Chang Gung Memorial Hospital, Taoyuan, Taiwan, between January 1986 and June 2015. They were treated according to the Taiwan Pediatric Oncology Group (TPOG) protocols. The clinical features and long-term outcomes were analyzed.
RESULTS: Six patients with histologically-proved bilateral WT were identified for analysis. One additional patient who was diagnosed with unilateral WT-associated intralobar nephrogenic rest, in addition to two small lesions in the contralateral kidney, was also included. There were total of three male patients and four female patients. The median follow-up period was 19 years (range 8-29 years). Five patients underwent initial biopsy and preoperative chemotherapy followed by surgery, whereas two patients underwent initial surgery followed by adjuvant chemotherapy. Local recurrence was found in two patients. The 8-year event-free survival and overall survival rates were 71.4% and 100%, respectively. Two patients developed advanced stage of chronic kidney disease, but none had been diagnosed with secondary malignant neoplasm. Other health issues such as hypertension, scoliosis and unspecified autoimmune disease were also found.
CONCLUSIONS: The treatment outcome in this study is comparably superior to other western countries. However, survivors of bilateral WT still have many chronic health issues and thereby need individualized long-term medical care.

Related: Kidney Cancer

Tüysüz G, Tayfun F, Canpolat F, et al.
A case of xanthogranulomatous pyelonephritis mimicking Wilms tumor.
Turk J Pediatr. 2015 Jul-Aug; 57(4):409-412 [PubMed] Related Publications
Xanthogranulomatous pyelonephritis (XGPN) is a very rare, unusual variant of pyelonephritis characterized by destruction of renal parenchyma. It usually occurs in adults with a history of recurrent urinary tract infections. The condition is rare in children and the disease can imitate renal tumors. Here, we describe a 12-year-old boy who presented with abdominal pain. He did not have any history of urinary tract infection. Computed tomography and magnetic resonance imaging showed a cystic lesion in the left upper kidney. The patient underwent radical nephrectomy with a provisional diagnosis of Wilms tumor however histopathological examination of specimen revealed XGPN. Xanthogranulomatous pyelonephritis should be kept in mind in the differential diagnosis of renal lesions in childhood, during surgery if any suspicion from the diagnosis, a frozen biopsy should have been taken.

Related: Kidney Cancer

Tipirneni-Sajja A, Loeffler RB, Oesingmann N, et al.
Measurement of glomerular filtration rate by dynamic contrast-enhanced magnetic resonance imaging using a subject-specific two-compartment model.
Physiol Rep. 2016; 4(7) [PubMed] Article available free on PMC after 01/01/2018 Related Publications
Measuring glomerular filtration rate (GFR) by dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI) as part of standard of care clinicalMRIexams (e.g., in pediatric solid tumor patients) has the potential to reduce diagnostic burden. However, enthusiasm for this relatively newGFRtest may be curbed by the limited amount of cross-calibration studies with referenceGFRtechniques and the vast variety ofMRtracer model algorithms causing confusion on the choice of model. To advanceMRI-basedGFRquantification via improvedGFRmodeling and comparison with associated(99m)Tc-DTPA-GFR, 29 long-term Wilms' tumor survivors (19.0-43.3 years, [median 32.0 ± 6.0 years]) treated with nephrectomy, nonnephrotoxic chemotherapy ± radiotherapy underwentMRIwith Gd-DTPAadministration and a(99m)Tc-DTPA GFRtest. ForDCE-MRI-basedGFRestimation, a subject-specific two-compartment (SS-2C) model was developed that uses individual hematocrit values, automatically defines subject-specific uptake intervals, and fits tracer-uptake curves by incorporating these measures. The association between reference(99m)Tc-DTPA GFRandMR-GFRs obtained bySS-2C, three published 2C uptake, and inflow-outflow models was investigated via linear regression analysis. Uptake intervals varied from 64 sec to 141 sec [96 sec ± 21 sec] and hematocrit values ranged from 30% to 49% [41% ± 4%]; these parameters can therefore not be assumed as constants in 2C modeling. OurMR-GFRestimates using theSS-2C model showed accordingly the highest correlation with(99m)Tc-DTPA-GFRs (R(2) = 0.76,P < 0.001) compared with other models (R(2)-range: 0.36-0.66). In conclusion,SS-2C modeling ofDCE-MRIdata improved the association betweenGFRobtained by(99m)Tc-DTPAand Gd-DTPA DCE-MRIto such a degree that this approach could turn into a viable, diagnosticGFRassay without radiation exposure to the patient.

Ludwig N, Werner TV, Backes C, et al.
Combining miRNA and mRNA Expression Profiles in Wilms Tumor Subtypes.
Int J Mol Sci. 2016; 17(4):475 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
Wilms tumor (WT) is the most common childhood renal cancer. Recent findings of mutations in microRNA (miRNA) processing proteins suggest a pivotal role of miRNAs in WT genesis. We performed miRNA expression profiling of 36 WTs of different subtypes and four normal kidney tissues using microarrays. Additionally, we determined the gene expression profile of 28 of these tumors to identify potentially correlated target genes and affected pathways. We identified 85 miRNAs and 2107 messenger RNAs (mRNA) differentially expressed in blastemal WT, and 266 miRNAs and 1267 mRNAs differentially expressed in regressive subtype. The hierarchical clustering of the samples, using either the miRNA or mRNA profile, showed the clear separation of WT from normal kidney samples, but the miRNA pattern yielded better separation of WT subtypes. A correlation analysis of the deregulated miRNA and mRNAs identified 13,026 miRNA/mRNA pairs with inversely correlated expression, of which 2844 are potential interactions of miRNA and their predicted mRNA targets. We found significant upregulation of miRNAs-183, -301a/b and -335 for the blastemal subtype, and miRNAs-181b, -223 and -630 for the regressive subtype. We found marked deregulation of miRNAs regulating epithelial to mesenchymal transition, especially in the blastemal subtype, and miRNAs influencing chemosensitivity, especially in regressive subtypes. Further research is needed to assess the influence of preoperative chemotherapy and tumor infiltrating lymphocytes on the miRNA and mRNA patterns in WT.

Related: Kidney Cancer MicroRNAs

Smits EL, Stein B, Nijs G, et al.
Generation and Cryopreservation of Clinical Grade Wilms' Tumor 1 mRNA-Loaded Dendritic Cell Vaccines for Cancer Immunotherapy.
Methods Mol Biol. 2016; 1393:27-35 [PubMed] Related Publications
First described in the 1970s, dendritic cells (DC) are currently subjects of intense investigation to exploit their unique antigen-presenting and immunoregulatory capacities. In cancer, DC show promise to elicit or amplify immune responses directed against cancer cells by activating natural killer (NK) cells and tumor antigen-specific T cells. Wilms' tumor 1 (WT1) protein is a tumor-associated antigen that is expressed in a majority of cancer types and has been designated as an antigen of major interest to be targeted in clinical cancer immunotherapy trials. In this chapter, we describe the generation, cryopreservation, and thawing of clinical grade autologous monocyte-derived DC vaccines that are loaded with WT1 by messenger RNA (mRNA) electroporation. This in-house-developed transfection method gives rise to presentation of multiple antigen epitopes and can be used for all patients without restriction of human leukocyte antigen (HLA) type.

Related: WT1

Pritchard-Jones K, Graf N, van Tinteren H, Craft A
Evidence for a delay in diagnosis of Wilms' tumour in the UK compared with Germany: implications for primary care for children.
Arch Dis Child. 2016; 101(5):417-20 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
The UK has a longstanding system of general practice which provides the vast majority of primary care, including that for children. It acts as a 'gatekeeper' to more specialist care. Parents may also use accident and emergency departments as their first point of medical contact for their children. Outcomes in the UK for many conditions in children appear to be worse than in comparable European countries where there is direct access to care by paediatricians. We have therefore looked at pathways to diagnosis and compared outcomes in the childhood kidney cancer, Wilms' tumour, which has been treated in the UK and Germany within the same clinical trial for over a decade. We find that Wilms' tumours are significantly larger in volume and have a more advanced tumour stage at diagnosis in the UK compared to Germany. There is a small (∼3%) difference in event free and overall survival between the two countries. Our data suggest that the system of primary care for children in the UK is less likely to result in the incidental finding of an abdominal mass in a child with no or vague symptoms. This may be a reason for the poorer outcome.

Related: Kidney Cancer

Huang L, Mokkapati S, Hu Q, et al.
Nephron Progenitor But Not Stromal Progenitor Cells Give Rise to Wilms Tumors in Mouse Models with β-Catenin Activation or Wt1 Ablation and Igf2 Upregulation.
Neoplasia. 2016; 18(2):71-81 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
Wilms tumor, a common childhood tumor of the kidney, is thought to arise from undifferentiated renal mesenchyme. Variable tumor histology and the identification of tumor subsets displaying different gene expression profiles suggest that tumors may arise at different stages of mesenchyme differentiation and that this ontogenic variability impacts tumor pathology, biology, and clinical outcome. To test the tumorigenic potential of different cell types in the developing kidney, we used kidney progenitor-specific Cre recombinase alleles to introduce Wt1 and Ctnnb1 mutations, two alterations observed in Wilms tumor, into embryonic mouse kidney, with and without biallelic Igf2 expression, another alteration that is observed in a majority of tumors. Use of a Cre allele that targets nephron progenitors to introduce a Ctnnb1 mutation that stabilizes β-catenin resulted in the development of tumors with a predominant epithelial histology and a gene expression profile in which genes characteristic of early renal mesenchyme were not expressed. Nephron progenitors with Wt1 ablation and Igf2 biallelic expression were also tumorigenic but displayed a more triphasic histology and expressed early metanephric mesenchyme genes. In contrast, the targeting of these genetic alterations to stromal progenitors did not result in tumors. These data demonstrate that committed nephron progenitors can give rise to Wilms tumors and that committed stromal progenitors are less tumorigenic, suggesting that human Wilms tumors that display a predominantly stromal histology arise from mesenchyme before commitment to a stromal lineage.

Related: IGF2 Kidney Cancer CTNNB1

Erginel B, Ugurlucan M, Basaran M, et al.
Management of a Wilms' tumor with intracardiac extension using extracorporeal circulation and deep hypothermic circulatory arrest: Case report and review of the literature.
Pediatr Hematol Oncol. 2016; 33(1):67-73 [PubMed] Related Publications
Wilms' tumor is a relatively common malignancy among childhood cancers. However, intracardiac extension of the lesion is rare and challenging. In this report, the authors present a successful management of intracardiac extension of Wilms' tumor in a 3-year-old child using cardiopulmonary bypass and deep hypothermic circulatory arrest. The authors also reviewed the published literature on Wilms' tumor with cardiac extension, which were managed by cardiopulmonary bypass and deep hypothermic circulatory arrest to provide an optimum management plan in this challenging condition.

Gupta AK, Charlton A, Prelog K, Kellie SJ
β-HCG Elevation in Wilms Tumor: An Uncommon Presentation.
Pediatr Blood Cancer. 2016; 63(6):1105-6 [PubMed] Related Publications
Wilms tumor (nephroblastoma) is a readily diagnosed common abdominal tumor in children. Rarely, it may present with factors that may confound the diagnosis. We report a 6-year-old female child who presented with a rapidly growing and invasive abdominal mass with the histopathologic features of Wilms tumor associated with an elevated serum beta human chorionic gonadotropin, which has not been previously reported in this condition.

Related: Kidney Cancer

Deng C, Dai R, Li X, Liu F
Genetic variation frequencies in Wilms' tumor: A meta-analysis and systematic review.
Cancer Sci. 2016; 107(5):690-9 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
Over the last few decades, numerous biomarkers in Wilms' tumor have been confirmed and shown variations in prevalence. Most of these studies were based on small sample sizes. We carried out a meta-analysis of the research published from 1992 to 2015 to obtain more precise and comprehensive outcomes for genetic tests. In the present study, 70 out of 5175 published reports were eligible for the meta-analysis, which was carried out using Stata 12.0 software. Pooled prevalence for gene mutations WT1, WTX, CTNNB1, TP53, MYCN, DROSHA, and DGCR8 was 0.141 (0.104, 0.178), 0.147 (0.110, 0.184), 0.140 (0.100, 0.190), 0.410 (0.214, 0.605), 0.071 (0.041, 0.100), 0.082 (0.048, 0.116), and 0.036 (0.026, 0.046), respectively. Pooled prevalence of loss of heterozygosity at 1p, 11p, 11q, 16q, and 22q was 0.109 (0.084, 0.133), 0.334 (0.295, 0.373), 0.199 (0.146, 0.252), 0.151 (0.129, 0.172), and 0.148 (0.108, 0.189), respectively. Pooled prevalence of 1q and chromosome 12 gain was 0.218 (0.161, 0.275) and 0.273 (0.195, 0.350), respectively. The limited prevalence of currently known genetic alterations in Wilms' tumors indicates that significant drivers of initiation and progression remain to be discovered. Subgroup analyses indicated that ethnicity may be one of the sources of heterogeneity. However, in meta-regression analyses, no study-level characteristics of indicators were found to be significant. In addition, the findings of our sensitivity analysis and possible publication bias remind us to interpret results with caution.

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Hillen LM, Kamsteeg EJ, Schoots J, et al.
Refining the Diagnosis of Congenital Nephrotic Syndrome on Long-term Stored Tissue: c.1097G>A (p.(Arg366His)) WT1 Mutation Causing Denys Drash Syndrome.
Fetal Pediatr Pathol. 2016; 35(2):112-9 [PubMed] Related Publications
Congenital nephrotic syndrome (CNS) caused by a mutation in the Wilms tumor 1 suppressor gene (WT1) is part of Denys Drash Syndrome or Frasier syndrome. In the framework of genetic counseling, the diagnosis of CNS can be refined with gene mutation studies on long-term stored formalin-fixed paraffin-embedded tissue from postmortem examination. We report a case of diffuse mesangial sclerosis with perinatal death caused by a de novo mutation in the WT1 gene in a girl with an XY-genotype. This is the first case of Denys Drash Syndrome with the uncommon missense c.1097G>A [p.(Arg366His)] mutation in the WT1 gene which has been diagnosed on long-term stored formalin-fixed paraffin-embedded tissue in 1993. This emphasizes the importance of retained and adequately stored tissue as a resource in the ongoing medical care and counseling.

Related: Denys-Drash Syndrome FISH WT1

Varma AV, Malukani K, Rihal P, Nandedkar SS
Adult Wilms' tumor: A case report with review of literature.
J Cancer Res Ther. 2015 Oct-Dec; 11(4):934-6 [PubMed] Related Publications
Wilms' tumor presents a diagnostic problem due to its rare occurrence in adults. Most of the cases of adult Wilms' tumor are diagnosed unexpectedly following nephrectomy for renal cell carcinoma. We are reporting herein a rare case of Adult Wilms' tumor of kidney with triphasic histology and distant metastasis to lung.

Related: Kidney Cancer

Cao X, Liu DH, Zhou Y, et al.
Histone deacetylase 5 promotes Wilms' tumor cell proliferation through the upregulation of c-Met.
Mol Med Rep. 2016; 13(3):2745-50 [PubMed] Related Publications
The histone deacetylase (HDAC) family is comprised of enzymes, which are involved in modulating the majority of critical cellular processes, including transcriptional regulation, apoptosis, proliferation and cell cycle progression. However, the biological function of HDAC5 in Wilms' tumor remains to be fully elucidated. The present study aimed to investigate the expression and function of HDAC5 in Wilm's tumor. It was demonstrated that the mRNA and protein levels of HDAC5 were upregulated in human Wilms' tumor tissues. Overexpression of HDAC5 in G401 cells was observed to significantly promote cellular proliferation, as demonstrated by the results of an MTT assay and bromodeoxyuridine incorporation assay. By contrast, HDAC5 knockdown using small interfering RNA suppressed the proliferation of the G401 cells. At the molecular level, the present study demonstrated that HDAC5 promoted the expression of c‑Met, which has been previously identified as an oncogene. In addition, downregulation of c‑Met inhibited the proliferative effects of HDAC5 in human Wilms' tumor cells. Taken together, these results suggested that HDAC5 promotes cellular proliferation through the upregulation of c‑Met, and may provide a novel therapeutic target for the treatment of patients with Wilms' tumor.

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Weaver MS, Federico S
Visual Diagnosis: New Lung Nodules in a 3-year-old Girl with Wilms Tumor.
Pediatr Rev. 2016; 37(2):e5-6 [PubMed] Related Publications
The most significant oncologic concern with finding new pulmonary nodules on imaging in a pediatric patient who has anaplastic Wilms tumor is progressive disease with new pulmonary metastases. This case emphasizes the importance of employing a creative clinical differential diagnosis, even for patients with known underlying oncologic disease.

Related: Kidney Cancer

Rabeh W, Akel S, Eid T, et al.
Wilms tumor: Successes and challenges in management outside of cooperative clinical trials.
Hematol Oncol Stem Cell Ther. 2016; 9(1):20-5 [PubMed] Related Publications
OBJECTIVE/BACKGROUND: Management of Wilms tumor (WT) in children depends on a multidisciplinary approach to treatment, and outcomes have significantly improved as reported by cooperative group clinical trials. Here, we review the clinical outcomes of patients with WT and identify challenges and barriers encountered in multidisciplinary management outside of cooperative clinical trials.
METHODS: We retrospectively reviewed the clinical records of 35 children with WT treated between April 2002 and June 2013 at the Children's Cancer Institute in Lebanon.
RESULTS: Upfront resection was performed in 23 cases. Biopsies were performed for Stage V tumors (n=4), those with unresectable tumors or inferior vena caval thrombus (n=5), and patients who had partial surgery performed elsewhere prior to presentation (n=2). One patient died due to toxicity prior to surgery. The tumor was Stage I in eight patients, Stage II in five patients, Stages III and IV in nine patients each, and bilateral (Stage V) in four patients. Adherence to The National Wilms Tumor Study-5 recommendations was adequate. At the time of analysis, 30 patients were free of disease and four patients had relapse-all having metastatic disease initially.
CONCLUSION: The National Wilms Tumor Study-5 therapy resulted in favorable outcomes in children with nonmetastatic Wilms tumor in the setting of a multidisciplinary approach to therapy and resolution of financial barriers to medical care. Upstaging due to prior intervention and lung radiation therapy to all those with computed tomography-detected lung nodules may both have resulted in overtreatment of a subset of patients. Finally, the relatively high incidence of bilateral tumors suggests the need for further genetic and molecular studies in this patient population.

Related: Kidney Cancer

Spreafico F, Ciceri S, Gamba B, et al.
Chromosomal anomalies at 1q, 3, 16q, and mutations of SIX1 and DROSHA genes underlie Wilms tumor recurrences.
Oncotarget. 2016; 7(8):8908-15 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
Approximately half of children suffering from recurrent Wilms tumor (WT) develop resistance to salvage therapies. Hence the importance to disclose events driving tumor progression/recurrence. Future therapeutic trials, conducted in the setting of relapsing patients, will need to prioritize targets present in the recurrent lesions. Different studies identified primary tumor-specific signatures associated with poor prognosis. However, given the difficulty in recruiting specimens from recurrent WTs, little work has been done to compare the molecular profile of paired primary/recurrent diseases. We studied the genomic profile of a cohort of eight pairs of primary/recurrent WTs through whole-genome SNP arrays, and investigated known WT-associated genes, including SIX1, SIX2 and micro RNA processor genes, whose mutations have been recently proposed as associated with worse outcome. Through this approach, we sought to uncover anomalies characterizing tumor recurrence, either acquired de novo or already present in the primary disease, and to investigate whether they overlapped with known molecular prognostic signatures. Among the aberrations that we disclosed as potentially acquired de novo in recurrences, some had been already recognized in primary tumors as associated with a higher risk of relapse. These included allelic imbalances of chromosome 1q and of chromosome 3, and CN losses on chromosome 16q. In addition, we found that SIX1 and DROSHA mutations can be heterogeneous events (both spatially and temporally) within primary tumors, and that their co-occurrence might be positively selected in the progression to recurrent disease. Overall, these results provide new insights into genomic and genetic events underlying WT progression/recurrence.

Related: Chromosome 1 Chromosome 16 Chromosome 3 Kidney Cancer DICER1

Arjmandi K, Rostami T, Yusefian S, et al.
Brain Metastasis in Wilms' Tumor: a Case Report.
Acta Med Iran. 2015; 53(11):731-2 [PubMed] Related Publications
Wilms' tumor is the most common abdominal tumor of childhood, and its cerebral metastasis is apparently very rare. The authors report an 18-month-old girl with Wilms' tumor and brain metastasis.

Related: Kidney Cancer

Szymanska A, Augustyn C, Stankowski T, et al.
Wilms' Tumor With Intra-Atrial Extension: Treatment and Management.
World J Pediatr Congenit Heart Surg. 2016; 7(1):116-9 [PubMed] Related Publications
Wilms' tumor is the most common renal cancer in children. It can grow for a long time without any characteristic symptoms, causing only fever, abdominal pain, nausea, or vomiting, which is the reason why it is often discovered accidentally. In 1% to 4% of the cases, nephroblastoma leads to complications in the form of intravascular and intra-atrial extension. We present a case of a five-year-old boy with Wilms' tumor extending into the inferior vena cava, right atrium, and then prolapsing through the tricuspid valve into the right ventricle.

Related: Kidney Cancer

Özyörük D, Demir HA, Emir S, et al.
Occurrence of Wilms' tumor in a child with hereditary spherocytosis.
Turk J Pediatr. 2015 Mar-Apr; 57(2):206-9 [PubMed] Related Publications
Hereditary spherocytosis (HS) is the most frequent cause of congenital hemolytic anemia. It is an autosomal dominant genetic disorder characterized by cell membrane abnormalities, specifically in red blood cells. Although the association between benign, borderline and malignant tumors and HS is not clear, various tumors such as splenoma, adrenal myolipoma, pancreatic schwannoma, ganglioneuroma, extramedullary hematopoiesis, myeloproliferative disorders, multiple myeloma, B-cell lymphoma and acute lymphoblastic leukemia have been presented in case reports concerning HS patients. Here we describe a 6-year-old boy with HS who presented with a mass in the left kidney. Tru-cut biopsy revealed Wilms' tumor (WT). To the best of our knowledge, this is the first case of WT associated with HS to be reported in the literature.

Related: Kidney Cancer

Perlman EJ, Gadd S, Arold ST, et al.
MLLT1 YEATS domain mutations in clinically distinctive Favourable Histology Wilms tumours.
Nat Commun. 2015; 6:10013 [PubMed] Article available free on PMC after 01/01/2018 Related Publications
Wilms tumour is an embryonal tumour of childhood that closely resembles the developing kidney. Genomic changes responsible for the development of the majority of Wilms tumours remain largely unknown. Here we identify recurrent mutations within Wilms tumours that involve the highly conserved YEATS domain of MLLT1 (ENL), a gene known to be involved in transcriptional elongation during early development. The mutant MLLT1 protein shows altered binding to acetylated histone tails. Moreover, MLLT1-mutant tumours show an increase in MYC gene expression and HOX dysregulation. Patients with MLLT1-mutant tumours present at a younger age and have a high prevalence of precursor intralobar nephrogenic rests. These data support a model whereby activating MLLT1 mutations early in renal development result in the development of Wilms tumour.

Related: Kidney Cancer MLLT1 MYC

Yu X, Li Z, Chan MT, Wu WK
The roles of microRNAs in Wilms' tumors.
Tumour Biol. 2016; 37(2):1445-50 [PubMed] Related Publications
Wilms' tumor is the most common renal tumor in children in which diffusely anaplastic or unfavorable histology foreshadows poor prognosis. MicroRNAs are small, non-coding RNAs that negatively regulate gene expression at the posttranscriptional level. Accumulating evidence shows that microRNA dysregulation takes part in the pathogenesis of many renal diseases, such as chronic kidney diseases, polycystic kidney disease, renal fibrosis, and renal cancers. In Wilms' tumor, dysregulation of some key oncogenic or tumor-suppressing microRNAs, such as miR-17~92 cluster, miR-185, miR-204, and miR-483, has been documented. In this review, we will summarize current evidence on the role of dysregulated microRNAs in the development of Wilms' tumor.

Related: Kidney Cancer MicroRNAs

Green DM
Considerations in the Diagnosis and Management of Pediatric Patients With Favorable Histology Wilms Tumor Who Present With Only Pulmonary Nodules.
Pediatr Blood Cancer. 2016; 63(4):589-92 [PubMed] Article available free on PMC after 01/04/2017 Related Publications
More than 70% of children with stage IV, favorable histology (FH) Wilms tumor will be relapse-free survivors 16 years after diagnosis. Successful treatment generally includes whole lung radiation therapy and doxorubicin. Such therapy is associated with adverse, long-term effects, including impaired pulmonary function, congestive heart failure, and second malignant neoplasms, especially breast cancer. Cooperative groups have adopted a risk-based approach to the treatment of these patients. It is important to recall the good overall prognosis for this group before recommendations for intensification are made based on preliminary data and in the absence of histological confirmation of persistent malignant disease.

Related: Kidney Cancer Lung Cancer

Geller JI
Current standards of care and future directions for "high-risk" pediatric renal tumors: Anaplastic Wilms tumor and Rhabdoid tumor.
Urol Oncol. 2016; 34(1):50-6 [PubMed] Related Publications
'High risk' renal tumors of childhood generally includes anaplastic Wilms tumor, rhabdoid tumor, and metastatic renal sarcomas and carcinomas. In this review, the epidemiology, biology, treatment and prognosis of anaplastic Wilms tumor and rhabdoid tumor are presented. Future directions related to management of such cancers are discussed, with insights provided into possible clinical trials in development that consider integration of novel targeted therapies.

Related: Kidney Cancer Malignant Rhabdoid Tumour

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