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Wilms tumour

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https://www.readbyqxmd.com/read/29779000/splenic-myoid-angioendothelioma-mimicking-metastatic-disease-in-an-8-year-old-with-stage-iv-wilms-tumour
#1
Adil A Shah, Luca Dragos, Timothy D Kane, Mikael Petrosyan
Myoid angioendothelioma are rare and benign vascular tumours of the spleen. Radiographic evaluation and diagnosis is often challenging and subjecting tissue samples to immuhistochemical analysis is often required to make a definitive diagnosis. Myoidangioendotheliomas can be managed with open or laparoscopic splenectomy with minimal risk of recurrent disease. Herein, we present a case of a myoid angioendothelioma in a patient with stage IV Wilms' tumour.
May 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29743143/the-wilms-tumour-1-gene-as-a-factor-in-non-syndromic-hypospadias-evidence-and-controversy
#2
REVIEW
Gergely Buglyó, Dániel Beyer, Sándor Biró, Éva Oláh
Hypospadias is one of the most frequent congenital anomalies of the male external genitalia. Its pathogenesis is due to largely unknown or poorly understood genetic factors and is further complicated by environmental-intrauterine-risk factors. One of the genes currently in focus by molecular biologists and clinicians studying syndromic forms of hypospadias is the Wilms' tumour 1 (WT1) gene. There is controversy over whether WT1 defects are also responsible for isolated hypospadias. In this review, we briefly cover the role of WT1 as a transcription factor and discuss proposed pathogenic pathways leading to hypospadias, outlining possible directions for research...
May 7, 2018: Pathology
https://www.readbyqxmd.com/read/29721059/cancer-stem-cells-are-regulated-by-stat3-signalling-in-wilms-tumour
#3
Yanmei Liu, Xuexiang Gao, Shuo Wang, Xuemin Yuan, Yunqing Pang, Jian Chen, Jing Wang
The survival rates associated with Wilms tumour (WT) remain dismal despite advancements in detection and treatment strategies. Cancer stem cells (CSCs) are correlated with the initiation, recurrence and metastasis of tumours, but its impact on Wilms cancer stem cell (WCSC) maintenance remains unclear. In this study, CD133+ cells were successfully isolated from a single-cell suspension of the G401 Wilms tumour cell line using magnetic activated cell sorting (MACS). Signal transducers and activators of transcription 3 (STAT3) has been implicated in tumorigenesis, but its contribution to the metastatic progression of WCSCs has not been investigated...
2018: Journal of Cancer
https://www.readbyqxmd.com/read/29705138/what-is-the-risk-of-local-recurrence-after-laparoscopic-transperitoneal-radical-nephrectomy-in-children-with-wilms-tumours-analysis-of-a-local-series-and-review-of-the-literature
#4
Aurore Bouty, Kate Burnand, Michael Nightingale, Annie Roberts, Marty Campbell, Mike O'Brien, Yves Heloury
BACKGROUND: To reduce long-term morbidity (adhesions-related complications and impaired quality of life due to scars), laparoscopy has been used as an alternative to open surgery in Wilms tumours (WTs). However, concerns have been raised on the risk of local recurrence after this type of resection. OBJECTIVE: The aim was to determine the incidence of local recurrence after laparoscopic transperitoneal radical nephrectomy (LTRN). STUDY DESIGN: We analysed 18 local cases and conducted a review of the English literature in Pubmed from 2004 to 2017 with the following keywords: (Wilms OR nephroblastoma) AND (laparoscopy OR minimally invasive surgery) AND 2004:3000...
April 12, 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29693799/evaluation-of-boost-irradiation-in-patients-with-intermediate-risk-stage-iii-wilms-tumour-with-positive-lymph-nodes-only-results-from-the-siop-wt-2001-registry
#5
Raquel Dávila Fajardo, Eva Oldenburger, Christian Rübe, Marta López-Yurda, Kathy Pritchard-Jones, Christophe Bergeron, Norbert Graf, Martine van Grotel, Harm van Tinteren, Daniel Saunders, Marry M van den Heuvel-Eibrink, Geert O Janssens, Foppe Oldenburger
OBJECTIVE: To evaluate the value of radiotherapy boost omission in patients with intermediate-risk, stage III Wilms tumours (WT) with positive lymph nodes (LN). METHODS AND MATERIALS: All patients with intermediate-risk, stage III (LN positive) WT consecutively registered in the SIOP-WT-2001 study were included in this analysis. Endpoints were 5-year event-free survival (EFS), loco-regional control (LRC) and overall survival (OS). RESULTS: Between June 2001 and May 2015, 2,569 patients with stage I to III WT after preoperative chemotherapy were registered in the SIOP-WT-2001 study...
April 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29560280/micro-rnas-are-involved-in-activation-of-epicardium-during-zebrafish-heart-regeneration
#6
Marcello Ceci, Claudia Carlantoni, Maria Azzurra Missinato, Davide Bonvissuto, Bruna Di Giacomo, Riccardo Contu, Nicla Romano
Zebrafish could be an interesting translational model to understand and improve the post-infarction trial and possible regeneration in humans. The adult zebrafish is able to regenerate efficiently after resecting nearly 20% of the ventricular apex. This process requires the concert activation of the epicardium and endocardium, as well as trans-differentiation of pre-existing cardiomyocytes that together replace the lost tissue. The molecular mechanisms involved in this activation process are not completely clarified...
2018: Cell Death Discovery
https://www.readbyqxmd.com/read/29515254/uncovering-and-deciphering-the-pro-invasive-role-of-hace1-in-melanoma-cells
#7
Najla El-Hachem, Nadia Habel, Tanesha Naiken, Hanene Bzioueche, Yann Cheli, Guillaume E Beranger, Emilie Jaune, Florian Rouaud, Nicolas Nottet, Frédéric Reinier, Céline Gaudel, Pascale Colosetti, Corine Bertolotto, Robert Ballotti
HACE1 is an E3 ubiquitin ligase described as a tumour suppressor because HACE1-knockout mice develop multi-organ, late-onset cancers and because HACE1 expression is lost in several neoplasms, such as Wilms' tumours and colorectal cancer. However, a search of public databases indicated that HACE1 expression is maintained in melanomas. We demonstrated that HACE1 promoted melanoma cell migration and adhesion in vitro and was required for mouse lung colonisation by melanoma cells in vivo. Transcriptomic analysis of HACE1-depleted melanoma cells revealed an inhibition of ITGAV and ITGB1 as well changes in other genes involved in cell migration...
March 7, 2018: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29459585/ultrasonographic-evaluation-of-malignant-pediatric-abdominal-tumour-with-histopathological-correlation
#8
M Hoshneara, M U Ahmed, M M Rahman, M Begum, R Ferdousi, U Ayma, K Begum, R Ara, M S Islam, I H Chowdhury, M A Sangma, M N Farzana
Ultrasonography is a very useful diagnostic tool to evaluate pediatric abdominal mass. This cross sectional study was conducted among clinically suspected patients having malignant abdominal mass attending in the department of Radiology & Imaging, Mymensingh Medical College Hospital, Mymensingh, Bangladesh from January 2008 to December 2009. Total 56 patients were included in this study. Patients were scanned by high resolution gray scale ultrasonography of the abdominal masses. After surgical procedure, biopsy specimen were collected in a container containing 10% formalin and sent for histopathological examination...
January 2018: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/29375007/recognition-mechanism-of-wilms-tumour-suppressor-protein-and-dna-triplets-insights-from-molecular-dynamics-simulation-and-free-energy-analysis
#9
Ling-Fei Zhang, Qing-Chuan Zheng, Hong-Xing Zhang
The Wilms' tumour suppressor protein (WT1) plays a multifaceted role in human cancer processes. Mutations on its DNA recognition domain could lead to Denys-Drash syndrome, and alternate splicing results in insertion of the tripeptide Lys-Thr-Ser (KTS) between the third and fourth zinc fingers (ZFs), leading to changes in the DNA-binding function. However, detailed recognition mechanisms of the WT1-DNA complex have not been explored. To clarify the mutational effects upon WT1 towards DNA binding at the atomic level, molecular dynamics simulations and the molecular mechanics/Poisson Boltzmann surface area (MM/PBSA) method were employed...
February 12, 2018: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/29350457/improved-outcome-at-end-of-treatment-in-the-collaborative-wilms-tumour-africa-project
#10
Trijn Israels, Vivian Paintsil, Dalida Nyirenda, Francine Kouya, Glenn Mbah Afungchwi, Peter Hesseling, Clara Tump, Gertjan Kaspers, Liz Burns, Ramandeep Singh Arora, George Chagaluka, Philippa Nana, Lorna Renner, Elizabeth Molyneux
BACKGROUND: The Collaborative Wilms Tumour (WT) Africa Project has implemented an adapted WT treatment guideline in sub-Saharan Africa as a multi-centre prospective clinical trial. A retrospective, baseline evaluation of end-of-treatment outcome was performed for a 2-year period prior to the introduction of this guideline. The collaborative project aims to reduce both treatment abandonment and death during treatment to less than 10% for improving survival. PROCEDURE: All participating centres obtained local Institutional Research Board (IRB) approval and implemented the adapted WT treatment guideline...
May 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29345578/searching-for-new-targets-and-treatments-in-the-battle-against-squamous-cell-carcinoma-of-the-head-and-neck-with-specific-focus-on-tumours-of-the-tongue
#11
Nicola Sgaramella, Xiaolian Gu, Linda Boldrup, Philip J Coates, Robin Fahraeus, Luigi Califano, Gianpaolo Tartaro, Giuseppe Colella, Lena Norberg Spaak, Adrian Strom, Torben Wilms, Lorenzo Lo Muzio, Giovanni Dell'Aversana Orabona, Mario Santagata, Lotta Loljung, Riccardo Rossiello, Karin Danielsson, Klas Strindlund, Sandra Lillqvist, Karin Nylander
Squamous cell carcinoma of the head and neck, SCCHN, is a heterogeneous group of tumours not only concerning site of origin but also regarding aetiology. The 5-year survival for the whole group of SCCHN tumours has not significantly improved over the last 20-25 years. Apart from tumour spread to lymph nodes, N status, gains and losses of specific chromosomes are the only factors shown to be independent prognostic markers for these tumours. Worldwide an increasing number of people ? 40 years are seen being affected by tongue SCC, the most common tumour within the SCCHN group...
January 16, 2018: Current Topics in Medicinal Chemistry
https://www.readbyqxmd.com/read/29329488/genetic-and-molecular-insights-into-genotype-phenotype-relationships-in-osteopathia-striata-with-cranial-sclerosis-oscs-through-the-analysis-of-novel-mouse-wtx-mutant-alleles
#12
Glenda Comai, Agnès Boutet, Kristina Tanneberger, Filippo Massa, Ana-Sofia Rocha, Aurelie Charlet, Clara Panzolini, Fariba Jian Motamedi, Robert Brommage, Wolfgang Hans, Thomas Funck-Brentano, Martin Hrabe de Angelis, Christine Hartmann, Martine Cohen-Solal, Jürgen Behrens, Andreas Schedl
The X-linked WTX/AMER1 protein constitutes an important component of the β-catenin destruction complex that can both enhance and suppress canonical β-catenin signaling. Somatic mutations in WTX/AMER1 have been found in a proportion of the pediatric kidney cancer Wilms' tumor. By contrast, germline mutations cause the severe sclerosing bone dysplasia osteopathia striata congenita with cranial sclerosis (OSCS), a condition usually associated with fetal or perinatal lethality in male patients. Here we address the developmental and molecular function of WTX by generating two novel mouse alleles...
January 12, 2018: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/29285297/identification-of-wt1-as-determinant-of-heptatocellular-carcinoma-and-its-inhibition-by-chinese-herbal-medicine-salvia-chinensis-benth-and-its-active-ingredient-protocatechualdehyde
#13
Ning Wang, Hor-Yue Tan, Yau-Tuen Chan, Wei Guo, Sha Li, Yibin Feng
Candidates from Chinese herbal Medicine might be preferable in drug discovery as the abundant experiences of traditional use usually hint the clinical efficacy. In this study, we screened the anti-tumour effect of several commonly used Chinese herbal Medicines on human hepatocellular carcinoma cells (HCC). We identified that Salvia chinensia Benth. (Shijianchuan in Chinese, SJC) exhibited prominent in vitro inhibition of HCC cells and suppressed the orthotopic growth of HCC in the liver of mice and repressed the lung metastasis of tumour cells...
December 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/29240258/dna-and-rna-binding-by-the-wilms-tumour-gene-1-wt1-protein-kts-and-kts-isoforms-from-initial-observations-to-recent-global-genomic-analyses
#14
REVIEW
Tove Ullmark, Giorgia Montano, Urban Gullberg
The Wilms' tumour gene 1 protein (WT1) is a zinc finger transcription factor found indispensable for foetal development. WT1 has also been implicated in the development of tumours in several organ systems, including acute myeloid leukaemia (AML). Four main WT1 isoforms come from 2 alternative splice events. One alternative splice results in the inclusion or exclusion of 3 amino acids, KTS, between zinc fingers 3 and 4 in the WT1 protein. The KTS insert has been extensively investigated due to the functional implications for DNA and RNA binding...
March 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29159834/linc00473-antagonizes-the-tumour-suppressor-mir-195-to-mediate-the-pathogenesis-of-wilms-tumour-via-ikk%C3%AE
#15
Shibo Zhu, Wen Fu, Liyu Zhang, Kai Fu, Jinhua Hu, Wei Jia, Guochang Liu
OBJECTIVES: Although dramatic improvements of overall survival has achieved in patients with favourable histology Wilms tumour, disease recurrence is still the main cause of cancer-related death in childhood. Long non-coding RNAs (lncRNAs) as oncogenes or tumour suppressors are dysregulated during carcinogenesis. However, the role of lncRNAs in the pathogenesis of Wilms tumour is unknown. Here, an lncRNA LINC00473 signature that functioned as oncogene was identified in Wilms tumour. METHODS: Wilms tumour (n = 15) and relative normal tissues were collected...
February 2018: Cell Proliferation
https://www.readbyqxmd.com/read/29089605/position-paper-rationale-for-the-treatment-of-wilms-tumour-in-the-umbrella-siop-rtsg-2016-protocol
#16
REVIEW
Marry M van den Heuvel-Eibrink, Janna A Hol, Kathy Pritchard-Jones, Harm van Tinteren, Rhoikos Furtwängler, Arnauld C Verschuur, Gordan M Vujanic, Ivo Leuschner, Jesper Brok, Christian Rübe, Anne M Smets, Geert O Janssens, Jan Godzinski, Gema L Ramírez-Villar, Beatriz de Camargo, Heidi Segers, Paola Collini, Manfred Gessler, Christophe Bergeron, Filippo Spreafico, Norbert Graf
The Renal Tumour Study Group of the International Society of Paediatric Oncology (SIOP-RTSG) has developed a new protocol for the diagnosis and treatment of childhood renal tumours, the UMBRELLA SIOP-RTSG 2016 (the UMBRELLA protocol), to continue international collaboration in the treatment of childhood renal tumours. This protocol will support integrated biomarker and imaging research, focussing on assessing the independent prognostic value of genomic changes within the tumour and the volume of the blastemal component that survives preoperative chemotherapy...
December 2017: Nature Reviews. Urology
https://www.readbyqxmd.com/read/29068437/paediatrics-integrating-genomics-to-dig-deeper-into-wilms-tumour-biology
#17
William Mifsud, Kathy Pritchard-Jones
No abstract text is available yet for this article.
December 2017: Nature Reviews. Urology
https://www.readbyqxmd.com/read/29059753/re-evidence-for-a-delay-in-diagnosis-of-wilms-tumour-in-the-uk-compared-with-germany-implications-for-primary-care-for-children
#18
Douglas A Canning
No abstract text is available yet for this article.
November 2017: Journal of Urology
https://www.readbyqxmd.com/read/29054136/combined-methylome-and-transcriptome-analysis-during-rat-hepatic-stellate-cell-activation
#19
Eva Christine Schumacher, Silke Götze, Claus Kordes, Vladimir Benes, Dieter Häussinger
Hepatic stellate cells (HSCs) are mesenchymal stem cells (MSCs) of the liver. They are unique among MSCs, since HSCs remain in a quiescent, retinoid-storing state in the normal liver but become activated after liver injury and contribute to tissue repair. The epigenetic mechanisms accompanying the transition of HSCs from a quiescent to an activated state are in the focus of the present study. We investigated the methylome and transcriptome during this process and observed profound changes. While the promoter methylation correlated negatively with gene expression, the gene-body methylation revealed no clear correlation...
December 15, 2017: Stem Cells and Development
https://www.readbyqxmd.com/read/29040332/the-developmental-programme-for-genesis-of-the-entire-kidney-is-recapitulated-in-wilms-tumour
#20
Ryuji Fukuzawa, Matthew R Anaka, Ian M Morison, Anthony E Reeve
Wilms tumour (WT) is an embryonal tumour that recapitulates kidney development. The normal kidney is formed from two distinct embryological origins: the metanephric mesenchyme (MM) and the ureteric bud (UB). It is generally accepted that WT arises from precursor cells in the MM; however whether UB-equivalent structures participate in tumorigenesis is uncertain. To address the question of the involvement of UB, we assessed 55 Wilms tumours for the molecular features of MM and UB using gene expression profiling, immunohistochemsitry and immunofluorescence...
2017: PloS One
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