Clinical characteristics and outcomes of children with WAGR syndrome and Wilms tumor and/or nephroblastomatosis: The 30-year SIOP-RTSG experience. Hol Janna A,Jongmans Marjolijn C J,Sudour-Bonnange Hélène,Ramírez-Villar Gema L,Chowdhury Tanzina,Rechnitzer Catherine,Pal Niklas,Schleiermacher Gudrun,Karow Axel,Kuiper Roland P,de Camargo Beatriz,Avcin Simona,Redzic Danka,Wachtel Antonio,Segers Heidi,Vujanic Gordan M,van Tinteren Harm,Bergeron Christophe,Pritchard-Jones Kathy,Graf Norbert,van den Heuvel-Eibrink Marry M, Cancer BACKGROUND:WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, and range of developmental delays) is a rare contiguous gene deletion syndrome with a 45% to 60% risk of developing Wilms tumor (WT). Currently, surveillance and treatment recommendations are based on limited evidence. METHODS:Clinical characteristics, treatments, and outcomes were analyzed for patients with WAGR and WT/nephroblastomatosis who were identified through International Society of Pediatric Oncology Renal Tumor Study Group (SIOP-RTSG) registries and the SIOP-RTSG network (1989-2019). Events were defined as relapse, metachronous tumors, or death. RESULTS:Forty-three patients were identified. The median age at WT/nephroblastomatosis diagnosis was 22 months (range, 6-44 months). The overall stage was available for 40 patients, including 15 (37.5%) with bilateral disease and none with metastatic disease. Histology was available for 42 patients; 6 nephroblastomatosis without further WT and 36 WT, including 19 stromal WT (52.8%), 12 mixed WT (33.3%), 1 regressive WT (2.8%) and 2 other/indeterminable WT (5.6%). Blastemal type WT occurred in 2 patients (5.6%) after prolonged treatment for nephroblastomatosis; anaplasia was not reported. Nephrogenic rests were present in 78.9%. Among patients with WT, the 5-year event-free survival rate was 84.3% (95% confidence interval, 72.4%-98.1%), and the overall survival rate was 91.2% (95% confidence interval, 82.1%-100%). Events (n = 6) did not include relapse, but contralateral tumor development (n = 3) occurred up to 7 years after the initial diagnosis, and 3 deaths were related to hepatotoxicity (n = 2) and obstructive ileus (n = 1). CONCLUSIONS:Patients with WAGR have a high rate of bilateral disease and no metastatic or anaplastic tumors. Although they can be treated according to existing WT protocols, intensive monitoring of toxicity and surveillance of the remaining kidney(s) are advised. LAY SUMMARY:WAGR syndrome (Wilms tumor, aniridia, genitourinary anomalies, and range of developmental delays) is a rare genetic condition with an increased risk of developing Wilms tumor. In this study, 43 patients with WAGR and Wilms tumor (or Wilms tumor precursor lesions/nephroblastomatosis) were identified through the international registry of the International Society of Pediatric Oncology Renal Tumor Study Group (SIOP-RTSG) and the SIOP-RTSG network. In many patients (37.5%), both kidneys were affected. Disease spread to other organs (metastases) did not occur. Overall, this study demonstrates that patients with WAGR syndrome and Wilms tumor can be treated according to existing protocols. However, intensive monitoring of treatment complications and surveillance of the remaining kidney(s) are advised. 10.1002/cncr.33304
    Maternal and perinatal characteristics, congenital malformations and the risk of wilms tumor: the ESTELLE study. Bauer Hélène,Rios Paula,Schleiermacher Gudrun,Valteau-Couanet Dominique,Bertozzi Anne-Isabelle,Thebaud Estelle,Gandemer Virginie,Pellier Isabelle,Verschuur Arnauld,Spiegel Alexandra,Notz-Carrere Anne,Bergeron Christophe,Orsi Laurent,Lacour Brigitte,Clavel Jacqueline Cancer causes & control : CCC PURPOSE:Wilms tumor (WT), or nephroblastoma, is an embryonic tumor that constitutes the most common renal tumor in children. Little is known about the etiology of WT. The aim of this study was to investigate whether maternal or perinatal characteristics were associated with the risk of WT. METHODS:The ESTELLE study is a national-based case-control study that included 117 cases of WT and 1,100 controls younger than 11 years old. The cases were children diagnosed in France in 2010-2011 and the controls were frequency matched with cases by age and gender. The mothers of case and control children responded to a telephone questionnaire addressing sociodemographic and perinatal characteristics, childhood environment, and lifestyle. Unconditional logistic regression models adjusted on potential cofounders were used to estimate the odds ratios (OR) and their confidence intervals (95% CI). RESULTS:High birth weight and the presence of congenital malformation were associated with WT (OR 1.9 [95% CI 1.0-3.7] and OR 2.5 [95% CI 1.1-5.8], respectively). No association with breastfeeding or folic acid supplementation was observed. CONCLUSIONS:Although potential recall bias cannot be excluded, our findings reinforce the hypothesis that high birth weight and the presence of congenital malformation may be associated with an increased risk of WT. Further investigations are needed to further elucidate the possible role of maternal characteristics in the etiology of WT. 10.1007/s10552-020-01288-y
    Wilms Tumor of the Ovary: Review of the Literature and Report of 2 Cases. Turashvili Gulisa,Fix Daniel J,Soslow Robert A,Park Kay J International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists Primary extrarenal Wilms tumor of the gynecologic tract is extremely rare with scattered case reports occurring in the ovary, uterine corpus and cervix. Only 9 cases of primary ovarian Wilms tumor have been reported to date. Here, we provide an extensive literature review and describe 2 patients with ovarian Wilms tumor: a 36-yr-old female (patient 1) and a 16-yr-old female (patient 2), both presenting with abdominal pain and suspected ovarian torsion. They were each found to have unilateral ovarian masses measuring >15 cm in size which were removed by unilateral salpingo-oophorectomy. Microscopically, the tumors exhibited the typical triphasic histology of Wilms tumor. In addition, the tumor from patient 1 contained elements of mature cystic teratoma, while an extensive rhabdomyosarcomatous component was identified in patient 2. Both tumors were diffusely and strongly positive for WT1 with variable staining for other biomarkers. The cases were diagnostically challenging and referred to our center for an expert opinion. Teratoid Wilms tumor in patient 1 is the second reported case of ovarian Wilms tumor arising in association with teratoma. Recognition of primary ovarian Wilms tumor requires a high index of suspicion and exclusion of other entities based on tumor morphology and immunohistochemical studies. 10.1097/PGP.0000000000000565
    Surgical Management of Wilms Tumor (Nephroblastoma) and Renal Cell Carcinoma in Children and Young Adults. Lopyan Natalie M,Ehrlich Peter F Surgical oncology clinics of North America This article reviews the epidemiology, pathophysiology, clinical presentation, and multimodality management of Wilms tumors and renal cell carcinoma in pediatric and young adults. Key renal Société Internationale d'Oncologie Pédiatrique and Children Oncology Group studies are presented. The article reviews the common staging systems and risk-adapted treatment strategies with particular attention to the surgical management. 10.1016/j.soc.2020.11.002
    The contribution of WTAP gene variants to Wilms tumor susceptibility. Ma Li,Hua Rui-Xi,Lin Huiran,Zhu Jinhong,Fu Wen,Lin Ao,Zhang Jiao,Cheng Jiwen,Zhou Haixia,Li Suhong,Zhuo Zhenjian,He Jing Gene Wilms tumor is the most frequently occurring pediatric renal malignancy. Wilms tumor suppressor-1-associated protein (WTAP) is a vital component of N6-methyltransferase complex involved in tumorigenesis. However, the roles of WTAP gene single nucleotide polymorphisms (SNPs) in Wilms tumor risk have not been clarified to date. We successfully genotyped three WTAP gene SNPs using TaqMan assay in 405 Wilms tumor patients and 1197 cancer-free controls of Chinese children. Odds ratios (ORs) and 95% confidence intervals (CIs) were applied to determine the effects of WTAP gene SNPs on Wilms tumor risk. Carriers of the rs1853259 G variant are less susceptible to developing Wilms tumor, with an adjusted OR of 0.78 (AG vs. AA: 95% CI = 0.61-0.995, P = 0.046). Single locus analysis of rs9457712 G > A and rs7766006 G > T, as well as the combined analysis of risk genotypes, failed to unveil an association with Wilms tumor risk, respectively. Stratified analysis of the three SNPs and their combined risk effects showed more significant relationships with Wilms tumor risk under certain subgroups. In all, we found weak evidence of the association between WTAP gene SNPs and the risk of Wilms tumor. Further replication studies with greater sample size and different ethnicities are necessary to verify our findings. 10.1016/j.gene.2020.144839
    Relapsed Wilms' tumor in pediatric patients: challenges in low- to middle-income countries-a single-center experience. Zekri Wael,Yacoub Dalia M,Ibrahim Asmaa,Madney Youssef Journal of the Egyptian National Cancer Institute BACKGROUND:Wilms' tumor (WT) affects one in 10,000 children and accounts for 5% of all childhood cancers. Although the overall relapse rate for children with WT has decreased to less than 15 %, the overall survival for patients with recurrent disease remains poor at approximately 50 %. The aim of the study to evaluate the outcome of relapsed Wilms' tumor pediatric patients treated at the National Cancer Institute (NCI), Egypt, between January 2008 and December 2015. RESULTS:One hundred thirty (130) patients diagnosed with WT during the study period, thirty (23%) patients had relapsed. The median follow up period was 22.3 months (range 3.6-140 months). The Overall Survival (OS) was 30.9% while the event-free survival (EFS) was 29.8% at a 5-year follow up period. Median time from diagnosis to relapse was 14.4 months. A second complete remission was attained in 18/30 patients (60%). The outcome of the 30 patients; 11 are alive and 19 had died. Three factors in our univariate analysis were prognostically significant for survival after relapse. The first was radiotherapy given after relapse (p = 0.012). The 5-year EFS and OS for the group that received radiotherapy were 41.9% versus 16.7% and 11.1% respectively for those that did not. The second was the state of lymph nodes among patients with local stage III (p = 0.004). Lastly, when risk stratification has been applied retrospectively on our study group, it proved to be statistically significant (p = 0.029). CONCLUSION:Among relapsed pediatric WT, radiotherapy improved survival at the time of relapse and local stage III with positive lymph nodes had the worst survival among other stage III patients. 10.1186/s43046-020-00032-6
    Late Recurrence of Wilms Tumor After 17 Years: A Case Report. Park Woo Young,Hong Kyung Taek,Ahn Hong Yul,Choi Jung Yoon,Kang Hyoung Jin,Park Sung Hye,Shin Hee Young Journal of pediatric hematology/oncology Wilms tumor is the most common renal malignancy in children. Most of Wilms tumor recurrences occur within 2 years of the first diagnosis. Relapse after 5 years after the first diagnosis is called "late recurrence" and is rare in Wilms tumor. There are few case reports or small series of late recurrence of Wilms tumor. Because of the rarity of late recurrence of Wilms tumor, there is no clear guideline for its management. We describe a case of late recurrence of Wilms tumor as a remote metastasis in the lung at 18 years after the first diagnosis and 17 years after the second remission, which was achieved by radiotherapy and high-dose chemotherapy with autologous stem cell rescue. After late recurrence, the patient was treated by surgery and adjuvant chemotherapy, and remained disease-free for 11 months. Several very late recurrences of Wilms tumor in the literature are reviewed. 10.1097/MPH.0000000000001473
    Wilms tumor: 15 years of experience at a children's hospital in Córdoba, Argentina. Seminara Claudia,Planells M Celia,Pogonza Ramón E,Morales Miriam, Archivos argentinos de pediatria The objective of this study was to describe the epidemiology, clinical presentation, treatment and nephrology follow-up of children with Wilms tumor. Data from 46 patients were collected. The clinical presentation occurred at a young age (< 40 months old), with initial symptoms of pain, abdominal mass, and fever. The prevalent histology type was mixed nephroblastoma. All patients received pre-surgery chemotherapy followed by, in most cases, unilateral nephrectomy. Patients with a high histological risk had a 7.2 relative risk of death (75 % confidence interval: 1.5-33.7) compared to the rest, and a 2.5 relative risk of recurrence (75 % confidence interval: 1.0-6.4). Disease-free survival at 5 years was 70 %. Once cancer treatment was completed, 80 % of patients maintained a stage-I kidney function. The most important prognostic factor was histology. These patients required a long-term nephrology follow-up. 10.5546/aap.2019.eng.263
    [Comparative evaluation of preoperation transcatheter arterial chemoembolization in children with advanced wilms tumor]. Jia X,Lai C,Pan H P,Zhou H C,Yang L,Fei Z H Zhonghua yi xue za zhi To evaluate the therapeutic effect and prognosis of preoperative transcatheter arterial chemoembolization (TACE), transcatheter arterial chemoembolization (TAIC), preoperative intravenous chemotherapy for children with advanced stage nephroblastoma. From January 2007 to December 2018, according to different treatment protocols before surgery, children with nephroblastoma were divided into 3 groups, which were TACE group (44 cases), TAIC group (7 cases) and intravenous chemotherapy group (9 cases) in Children's Hospital, Zhejiang University School of Medicine. The imaging examination, treatment safety and long-term efficacy of these three groups before and after treatment were compared. Observed indicators include tumor debulking rate, envelope integrity rate, necrosis rate, postoperative adverse reactions and follow-up. Tumor debulking rate: tumor of TACE group decreased 46.5%, TAIC group shrinked 28.3%, and intravenous chemotherapy group reduced by 23.3%, the difference of tumor shrinkage between TACE group and intravenous chemotherapy group was statistically significant (0.05). Tumor necrosis rate: necrotic area in TACE group was about 46.0%-95.4%, average 75.1%±12.5%, while in intravenous chemotherapy group was 65.8%±8.7%, the difference was statistically significant (<0.01). The tumor membrane integrity rate of these three groups were 86.4%(38/44),5/7 and 6/9 respectively, and difference between TACE group and intravenous chemotherapy group was significant (<0.05). Patients who had myelosuppression each was 5 in TACE group (5/44), 4 in TAIC group (4/7), and 8 in intravenous chemotherapy group (8/9), and there were significant differences. Follow-up time and tumor-free survival rate in each group were respectively 20 to 92 months (median time 64 months) and 95% in TACE group, 12 to 69 months (median time 30 months) and 43.0% in TAIC group, and 16 to 72 months (median 28 months) and 56.0% in intravenous chemotherapy group. Preoperative TACE can lead to tumor shrinkage and necrosis more obviously, systemic adverse reactions are small, also the tumor complete resection rate is higher and the operation is safer.The survival rate can be effectively improved and more suitable for clinical treatment. 10.3760/cma.j.issn.0376-2491.2019.15.006
    Wilms tumor, medulloblastoma, and rhabdomyosarcoma in adult patients: lessons learned from the pediatric experience. Spreafico Filippo,Ferrari Andrea,Mascarin Maurizio,Collini Paola,Morosi Carlo,Biasoni Davide,Biassoni Veronica,Schiavello Elisabetta,Gandola Lorenza,Gattuso Giovanna,Chiaravalli Stefano,Massimino Maura Cancer metastasis reviews Wilms tumor (or nephroblastoma), rhabdomyosarcoma, and medulloblastoma, common embryonal tumors in children, can occasionally occur in adults, for whom survival is significantly inferior than pediatric patients. Available data on adults with Wilms tumor consist of case or case series reports. Among other factors, the unfamiliarity of adult oncologists and pathologists with nephroblastoma and consequent delays in initiating the appropriate risk-adapted chemotherapy may negatively influence outcomes. The survival decrement in adults with rhabdomyosarcoma has been attributed to the lack of centralized care, the inconsistent use of standard protocol-driven multimodal therapy, and lower chemotherapy tolerance in adult patients. In children with medulloblastoma, evidence from randomized clinical trials has led to risk-tailored therapies tuned on histology, extent of initial disease, and biological features. Such refinements are still missing for adults due to the lack of similar trials and studies that might provide the same or a different understanding regarding patients' individual prognosis, treatment morbidity, and quality of life. Recent experiences have suggested that applying or adjusting pediatric protocols to adult patients with these tumors is feasible and can improve survival. Here, we provide an evaluation of the current evidence for the management of Wilms tumor, rhabdomyosarcoma, and medulloblastoma arising in adults. This review aims to promote the referral of adolescents and adults with pediatric tumors to pediatric centers for inclusion into pediatric protocols, or into protocols and studies specifically designed for that age group with the cooperation between pediatric and adult oncologists. 10.1007/s10555-019-09831-3
    Immune expression in children with Wilms tumor: a pilot study. Holl E K,Routh J C,Johnston A W,Frazier V,Rice H E,Tracy E T,Nair S K Journal of pediatric urology BACKGROUND:Given improvements in multimodality therapy, survival among children with Wilms tumor (WT) exceeds 90%. However, 15% of children with favorable histology and 50% of children with anaplastic WT experience recurrence or progression. Of patients with advanced disease, only 50% survive to adulthood. In adult malignancies (including renal tumors), patient survival has improved with the advent of immunotherapy. However, little is known about the immune microenvironment of WT, making the potential role of immunotherapy unclear. OBJECTIVE:The objective of the study is to perform an exploratory, descriptive analysis of the immune milieu in WT. STUDY DESIGN:Between 2016 and 2017, all pediatric patients with WT, some of whom received neoadjuvant chemotherapy, underwent ex vivo wedge biopsy at the time of nephrectomy. The fresh tumor tissue and peripheral blood samples were analyzed for infiltrating immune infiltrate and effector cells using flow cytometry. Immunohistochemistry was performed for CD4, CD8, and PD-L1 expression. Matched blood samples were obtained for each patient, and circulating immune cells were analyzed by flow cytometry. RESULTS:A total of six patients were enrolled. One patient with neuroblastoma was excluded. The remaining five patients included the following: two with unilateral WT (resected before chemotherapy), two with bilateral WT (resected after neoadjuvant chemotherapy), and one with Denys-Drash syndrome, end-stage renal disease, and history of WT in the contralateral kidney. Immune analysis showed that WT were infiltrated by immune cells regardless of chemotherapy status. CD8 and CD4 T cells were present in the tumor tissue and exhibited an activated phenotype. Elevated levels of natural killer (NK) cells were observed in the tumors (Figure). Immune checkpoint PD-L1 was also found expressed in one of the tumors stained. DISCUSSION:In this pilot study, it was found that WTs were infiltrated by immune cells (CD45+) both before and after chemotherapy. Elevated levels of NK cells infiltrating the tumor specimens, which were quantitatively increased compared with levels of NK cells circulating in the blood, were noted. T cells, particularly CD4+ and CD8+ T cells, were present in tumor specimens. Tumor-infiltrating CD4 and CD8 T cells displayed an activated phenotype as defined by increased expression of human leukocyte antigen-DR isotype (HLA-DR), programmed cell death protein 1 (PD1), and CD57. Together, these findings suggest that WT microenvironment is immune engaged and may be susceptible to immunotherapy similar to other malignancies. CONCLUSIONS:These pilot data suggest an immune-engaged tumor microenvironment is present within WT. This implies that WT may be susceptible to immunotherapy similar to adult renal tumors and other adult malignancies. Follow-up studies are currently underway. 10.1016/j.jpurol.2019.03.011
    Comprehensive Analysis of lncRNA-Mediated ceRNA Crosstalk and Identification of Prognostic Biomarkers in Wilms' Tumor. Zheng Hong,Li Bai-Hui,Liu Chang,Jia Li,Liu Feng-Ting BioMed research international Wilms' tumor (WT) is the most common type of childhood kidney cancer, and most cases present with favorable histology and respond well to standard treatment. However, a subset of patients with WT is diagnosed with bilateral, relapsed, and high-risk tumors which remain the leading cause of cancer-related death in children. Long noncoding RNAs (lncRNAs) and their aberrant expression have currently been attracting great attention as oncogenes or tumor suppressors during tumor initiation and progression. So far, their roles and related competitive endogenous RNA (ceRNA) network remain unelucidated in nephroblastoma pathogenesis. We comprehensively integrated lncRNA, microRNA (miRNA), and messenger RNA (mRNA) expression profiles from the Therapeutically Applicable Research to Generate Effective Treatment (TARGET) database and screened out differentially expressed mRNAs (DEMs), lncRNAs (DELs), and miRNAs (DEMis) to construct a ceRNA network based on the information generated from miRcode, miRTarBase, TargetScan, and miRDB. Gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analyses were performed to analyze the functional characteristics of DEMs in the ceRNA network. The interaction between protein molecules was also analyzed by establishing a protein-protein interaction network. Finally, prognosis-related biomarkers were identified via survival analysis. Initially, 1647 DELs, 115 DEMis, and 3280 DEMs (|log FC| > 2; FDR < 0.01) were obtained using the R package. Next, we constructed a lncRNA-miRNA-mRNA network (ceRNA network), in which 176 DELs, 24 DEMis, and 141 DEMs were identified. Furthermore, 148 functional enrichment terms from GO were identified and 29 KEGG pathways were found to be significantly enriched. We also integrated patient clinical information to analyze the association between DERNAs and patient prognosis. We found that high expression of 8 DELs (LINC00473, AL445228.2, DENND5B-AS1, DLEU2, AC123595.1, AC135178.1, LINC00535, and LMO7-AS1) and 4 DEMs (CEP55, DEPDC1, PHF19, and TRIM36) correlated with poor survival in a patient with WT, whereas high expression of 2 DELs (MEG3 and RMST), 1 DEM (KIAA0922), and 1 DEMi (hsa-mir-200a) could possibly lead to better clinical outcomes. For the first time, the present study provided a novel insight into lncRNA-related ceRNA networks and identified potential prognostic biomarkers in Wilms' tumor. 10.1155/2020/4951692
    Association between different levels of lipid metabolism‑related enzymes and fatty acid synthase in Wilms' tumor. Wang Xiaoqing,Du Guoqiang,Wu Yidi,Zhang Yongfei,Guo Feng,Liu Wei,Wu Rongde International journal of oncology Wilms' tumor is one of the most common malignant tumors of the abdomen in children. However, there is currently no recognized specific biomarker for the clinical diagnosis and prognosis of this tumor. Lipid metabolism is involved in membrane synthesis and oxidation in tumor cells. This process plays an important role in the development of tumors, but it has not yet been investigated in Wilms' tumor. The aim of the present study was to characterize the changes in lipid metabolism and to contribute to the diagnosis and prognosis of Wilms' tumor. Proteomics analysis was performed to detect lipid‑metabolizing enzymes in 9 tissue samples from Wilms' tumors and adjacent tissues, and proteomics revealed the presence of 19 differentially expressed lipid‑metabolizing enzymes. Protein interaction analysis with the Search Tool for the Retrieval of Interacting Genes/Proteins was used to identify the interacting proteins. Immunohistochemistry (IHC), immunofluorescence and western blotting were used to further confirm whether the expression of fatty acid synthase (FASN) was significantly increased in the tumor tissues. Oncomine database and reverse transcription‑PCR analyses further confirmed that the expression of FASN at the gene level was significantly increased in the tumors. Following collection of 65 pediatric cases of Wilms' tumor at the Shandong Provincial Hospital between 2007 and 2012, the association between the expression of FASN and the clinical characteristics was analyzed, and IHC analysis further demonstrated that FASN expression was significantly associated with tumor stage and size. The association between FASN and the prognosis of children with Wilms' tumor was analyzed using Kaplan‑Meier survival curves. In addition, univariate survival analysis revealed that higher expression of FASN in Wilms' tumors was associated with poorer prognosis. Our findings revealed that FASN may be used as a prognostic biomarker in patients with Wilms' tumor. Furthermore, lipid metabolism may play an important role in the occurrence and development of Wilms' tumor. 10.3892/ijo.2019.4948
    Nephrectomy in children with wilms' tumor: 15 years of experience with "Tumor Delivery Technique". Mor Yoram,Zilberman Dorit Esther,Morag Roy,Ramon Jacob,Churi Chaim,Avigad Itamar African journal of paediatric surgery : AJPS Background:The contemporary surgical approach to Wilms' tumors follows that used in adults with renal cell carcinomas, namely, early occlusion of the renal vessels and then removal of the kidney as an intact mass. For years, the surgical approach at our institution has been different, starting with blunt separation of the kidney from the surrounding tissues, followed by its delivery outside the abdominal cavity while it is only attached to the major blood vessels which are subsequently ligated. We aimed to present this "tumor delivery technique" and evaluate its outcomes. Materials and Methods:We retrospectively reviewed medical records of children who underwent nephrectomy for Wilms' tumor using "tumor delivery technique." All procedures were performed by the same team, according to the same surgical principles. Results:Between 2000 and 2015, 36 children were operated. Median age was 31 months (interquartile range [IQR]: 6-45 mo), and median maximal tumor diameter was 10 cm (IQR: 8-13.9 cm). Tumors were located to the right side in 47%, left side in 42%, and bilateral in 11%. Twelve children have received preoperative neoadjuvant chemotherapy. Capsular disruption and tumor spillage were documented in 4 cases (11%). Conclusions:"Tumor delivery technique" is an easy and safe approach which might reduce the overall complication rates and the incidence of intraoperative tumor spillage. 10.4103/ajps.AJPS_113_16
    Screening and identification of non-inflammatory specific protein markers in Wilms' tumor tissues. Zhang Junjie,Guo Fei,Wang Lei,Zhao Wei,Zhang Da,Yang Heying,Yu Jiekai,Niu Lili,Yang Fuquan,Zheng Shu,Wang Jiaxiang Archives of biochemistry and biophysics Wilms' tumor is one of the most common malignancies in children, and early diagnosis is critical for its subsequent treatment and prognosis. Our previous study employed proteomics to investigate protein markers in the serum of Wilms' tumor children. The present study aimed to identify specific protein markers in Wilms' tumor. Proteomic comparison of Wilms' tumor with normal kidney tissues and the sera of systemic inflammatory response syndrome (SIRS) controls was performed. Surface-enhanced laser desorption ionization time-of-flight (SELDI-TOF-MS) identified a protein with m/z 8350 as specific to Wilms' tumor. The target protein was purified using sodium dodecylsulfate polyacrylamide gel electrophoresis (SDS-PAGE) and identified as profilin-1 by matrix-assisted laser desorption/ionization time-of-flight/time-of-flight (MALDI-TOF/TOF). Its expression was validated using real-time polymerase chain reaction (RT-PCR) and enzyme-linked immunosorbent assay (ELISA). Our data identify profilin-1 as a potential protein marker for Wilms' tumor and demonstrate the feasibility of the above procedures for screening and identification of tumor-specific protein markers. 10.1016/j.abb.2019.108112
    The association of miR34b/c and TP53 gene polymorphisms with Wilms tumor risk in Chinese children. Wang Juxiang,Lou Susu,Huang Xiaokai,Mo Yixiao,Wang Zhen,Zhu Jinhong,Tian Xiaoqian,Shi Jiandong,Zhou Haixia,He Jing,Ruan Jichen Bioscience reports Wilms tumor is the most common pediatric malignancy in the kidney. The miR34b/c is a downstream target gene of the transcription factor p53. The important role of TP53 mutations, the methylation of miR34b/c, and the interaction between these two molecules in tumorigenesis have been well documented. Due to the biological connection between p53 and miR34b/c, in the present study, we investigated the association between polymorphisms in these two molecules and Wilms tumor susceptibility through genotyping two important functional polymorphisms (miR34b/c rs4938723 T>C and TP53 rs1042522 C>G) in 183 cases and 603 controls. Adjusted odds ratios (ORs) and 95% confidence intervals (CIs) derived from the logistic regression analysis were used to assess the correlation of miR34b/c rs4938723 and TP53 rs1042522 polymorphisms with Wilms tumor risk. Our results indicated that the association of miR34b/c rs4938723 and TP53 rs1042522 polymorphisms with Wilms tumor susceptibility was not statistically significant. Stratified analysis by age, gender, and clinical stage, as well as combined effect analysis were also performed, yet, no significant association was found. In conclusion, our study indicated a lack of association between the two selected polymorphisms and Wilms tumor susceptibility. Our findings need to be verified in studies with larger sample size in the future. 10.1042/BSR20194202
    MYCN gene polymorphisms and Wilms tumor susceptibility in Chinese children. Huang Xiaokai,Zhao Jie,Zhu Jinhong,Chen Shanshan,Fu Wen,Tian Xiaoqian,Lou Susu,Ruan Jichen,He Jing,Zhou Haixia Journal of clinical laboratory analysis BACKGROUND:Wilms tumor, derived from embryonic cells, accounts for a large proportion of pediatric renal tumors. MYCN encoded by MYCN proto-oncogene, a member of the MYC family, is a BHLH transcription factor. It plays a critical role in tumorigenesis and predicts poor clinical outcomes in various types of cancer. However, the role of MYCN remained unclarified in Wilms tumor. In this study, we investigated the association between MYCN gene polymorphisms and Wilms tumor susceptibility. METHODS:Four MYCN gene polymorphisms (rs57961569 G > A, rs9653226 T > C, rs13034994 A > G, and rs60226897 G > A) were genotyped in 183 cases and 603 controls. Adjusted odds ratios (AORs) and 95% confidence intervals (CIs) were calculated to evaluate the association between MYCN gene polymorphisms and Wilms tumor susceptibility. RESULTS:Overall, no significant association was found for any of the four MYCN gene polymorphisms. Interestingly, in the stratification analysis, the rs57961569 was found to be associated with decreased Wilms tumor susceptibility in the children older than 18 months (AOR = 0.65, 95% CI = 0.42-1.00, P = .050). Moreover, older children carrying 2-4 risk genotypes were at increased risk of Wilms tumor (OR = 1.55, 95% CI = 1.001-2.40, P = .0497). Haplotype GCAA was shown to significantly increased Wilms tumor risk (AOR = 2.40, 95% CI = 1.12-5.14, P = .024). CONCLUSION:Our study demonstrated that these MYCN gene polymorphisms might be low penetrant variants in Wilms tumor. 10.1002/jcla.22988
    Global Disparities in Wilms Tumor. Cunningham Megan E,Klug Theodore D,Nuchtern Jed G,Chintagumpala Murali M,Venkatramani Rajkumar,Lubega Joseph,Naik-Mathuria Bindi J The Journal of surgical research BACKGROUND:Wilms tumor accounts for more than 90% of all malignant kidney neoplasms in children. Survival after diagnosis and treatment is excellent in most high-income countries. Low- and middle-income countries (LMICs) continue to struggle with Wilms tumor detection and treatment. The purpose of this study was to compare the global incidence and outcomes of Wilms tumor. MATERIAL AND METHODS:Wilms tumor incidence data from the World Health Organization (WHO), International Incidence of Childhood Cancer, Volume III, was analyzed according to world region and country socioeconomic status using descriptive statistics and independent-sample Kruskal-Wallis Test. A literature review was also performed to assess outcomes and identify common themes. RESULTS:Wilms tumor was most common in children aged 0-4 y (median incidence 15.1 [IQR 11.8-18.7] ASR/million). High-income countries reported significantly higher median incidence than middle-income countries (8.6 [7.4-9.3] versus 6.1 [4.9-8.7] ASR/million; P < 0.01), although low-income countries reported the highest median incidence overall (9.8 [6.2-16.4] ASR/million). Low-income countries had the fewest countries with registries (n = 6). Overall survival ranged from 70% to 97% in high-income countries, 61%-94% in upper-middle-income countries, 0%-85% in lower-middle-income countries, and 25%-53% in low-income countries. Delay in diagnosis, lack of available treatment, and inadequate follow up contributed to the large variations in outcomes. CONCLUSIONS:Reported Wilms tumor incidence is highest in low-income countries, and these are also the countries that have the lowest survival. Lack of significance may reflect incomplete and absent data reporting from lower income countries. Accurate and comprehensive registries are the first steps to appropriate resource allocation in order to improve outcomes for this highly curable childhood malignancy. 10.1016/j.jss.2019.10.044
    Outcome of Children with Wilms' Tumor in Developing Countries. Bahoush Gholamreza,Saeedi Elahe Journal of medicine and life Wilms' tumor is the most common kidney tumor of childhood. The outcome of this malignant tumor has improved due to the improvement of therapeutic strategies. The most important factor in determining the prognosis of these patients is the histopathology subtype of the tumor; unfavorable histopathology is seen in only 11.5% of the patients, which accounts for 52% of deaths. Therefore, the aim of this study was to determine the outcome of children with Wilms' tumor referred to our hospital over a period of 10 years. This is a retrospective cohort study, and the target population included all patients with Wilms' tumor referred to Ali Asghar Hospital and were treated according to the National Wilms tumor study 4 (NWTS-4) protocol. All patients' data were extracted from the medical records of the department. Overall survival and event-free survival (EFS) were analyzed by the Kaplan Mayer method in the SPSS software, version 23. Fifty-two patients (24 male and 28 female patients) with Wilms' tumor were included. The mean age of the subjects was 40 months. The most common stage among boys and girls was stage II (23.08% and 28.85%, respectively). Our findings revealed that the overall five-year survival of patients was 87±5%; this figure was determined as 100% for boys and 76.8% ± 1.6 for girls (P = 0.018). Our findings show a dramatic improvement in the outcome of children with Wilms's tumor, and our results are comparable with other results from developed countries. Gender may be an independent prognostic factor of children with Wilms' tumor. 10.25122/jml-2019-0084