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wilms tumor review

Roberto Iglesias Lopes, Jessica Ming, Martin A Koyle, Ronald Grant, Adriana Fonseca, Armando J Lorenzo
OBJECTIVE: To describe the experience and technique of zero-ischemia laparoscopic-assisted partial nephrectomy at The Hospital for Sick Children, as an alternative to the traditional open approach for nephron sparing surgery in selected children with Wilms' tumors (WT). MATERIALS AND METHODS: Patients with diagnosis of WT treated with neoadjuvant chemotherapy and who underwent laparoscopic-assisted nephron-sparing surgery at the Hospital for Sick Children from 2012-2016 were identified and charts reviewed retrospectively...
October 6, 2016: Urology
Ariadne H A G Ooms, Samantha Gadd, Daniela S Gerhard, Malcolm A Smith, Jaime M Guidry Auvil, Daoud Meerzaman, Qing-Rong Chen, Chih Hao Hsu, Chunhua Yan, Cu Nguyen, Ying Hu, Yussanne Ma, Zusheng Zong, Andrew J Mungall, Richard A Moore, Marco A Marra, Vicki Huff, Jeffrey S Dome, Yueh-Yun Chi, Jing Tian, James I Geller, Charles G Mullighan, Jing Ma, David A Wheeler, Oliver A Hampton, Amy L Walz, Marry M van den Heuvel-Eibrink, Ronald R de Krijger, Nicole Ross, Julie M Gastier-Foster, Elizabeth J Perlman
PURPOSE: To investigate the role and significance of TP53 mutation in diffusely anaplastic Wilms tumors (DAWTs). EXPERIMENTAL DESIGN: All DAWTs registered on National Wilms Tumor Study-5 (n = 118) with available samples were analyzed for TP53 mutations and copy loss. Integrative genomic analysis was performed on 39 selected DAWTs. RESULTS: Following analysis of a single random sample, 57 DAWTs (48%) demonstrated TP53 mutations, 13 (11%) copy loss without mutation, and 48 (41%) lacked both [defined as TP53-wild-type (wt)]...
October 4, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Benoit Vaillancourt, Luc Laurier Oligny, Julie Déry, Julie Franc-Guimond, Dorothee Bouron-Dal Soglio
Ossifying renal tumor of infancy (ORTI) is a rare, benign pediatric tumor of the kidney. Since first reported by Chatten in 1980, 23 cases have been published. Previous authors have argued that ORTI might originate from nephrogenic rests, thereby sharing a pathogenic relationship with Wilms' tumor (WT). ORTI is characterized histologically by a population of polygonal osteoblast-like cells around an osteoid core and densely cellular component of blastemal-like or spindle cells. While the immunohistochemical profile of the cellular components has been reported, to the best of our knowledge the status of WT1 expression has only been reported once, where it showed negative marking...
August 30, 2016: Pediatric and Developmental Pathology
Jennifer Vogel, Haibo Lin, Stefan Both, Zelig Tochner, Frank Balis, Christine Hill-Kayser
BACKGROUND: Multimodality treatment for patients with Wilms tumor has improved patient survival, but is associated with acute and long-term toxicity, partially due to irradiation. Proton therapy using pencil beam scanning (PBS) is a promising technique to reduce dose to organs at risk (OAR). In this study, we evaluate PBS plans for postoperative irradiation in patients with Wilms tumor. PROCEDURE: Patients were treated with anterior-posterior-posterior-anterior (AP-PA) photon fields encompassing the preoperative tumor volume...
August 27, 2016: Pediatric Blood & Cancer
Ebubekir Dirican, Mustafa Akkiprik
The gene encoding Sal-like 4 (Drosophila) (SALL4) is a zinc-finger transcriptional factor and a vertebrate orthologous of the Drosophila gene spalt (sal), which is upregulated in some cancers. SALL4 is expressed in the early developmental stages of Drosophila. Moreover, murine SALL4 plays a vital role in protecting the properties of embryonic stem (ES) cells and guiding the outcome of the primal inner cell mass by interacting with OCT4 and NANOG. SALL4 in ES cells and tumor cells is known as a regulator for controlling cell growth, proliferation, and apoptosis...
July 21, 2016: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
Bettina Wilm, Ramon Muñoz-Chapuli
The Wilms' tumor suppressor gene 1 (Wt1) is critically involved in a number of developmental processes in vertebrates, including cell differentiation, control of the epithelial/mesenchymal phenotype, proliferation, and apoptosis. Wt1 proteins act as transcriptional and post-transcriptional regulators, in mRNA splicing and in protein-protein interactions. Furthermore, Wt1 is involved in adult tissue homeostasis, kidney function, and cancer. For these reasons, Wt1 function has been extensively studied in a number of animal models to establish its spatiotemporal expression pattern and the developmental fate of the cells expressing this gene...
2016: Methods in Molecular Biology
Alessandro Mussa, Cristina Molinatto, Giuseppina Baldassarre, Evelise Riberi, Silvia Russo, Lidia Larizza, Andrea Riccio, Giovanni Battista Ferrero
OBJECTIVE: To compare tumor risk in the 4 Beckwith-Wiedemann syndrome (BWS) molecular subgroups: Imprinting Control Region 1 Gain of Methylation (ICR1-GoM), Imprinting Control Region 2 Loss of Methylation (ICR2-LoM), Chromosome 11p15 Paternal Uniparental Disomy (UPD), and Cyclin-Dependent Kinase Inhibitor 1C gene (CDKN1C) mutation. STUDY DESIGN: Studies on BWS and tumor development published between 2000 and 2015 providing (epi)genotype-cancer correlations with histotype data were reviewed and meta-analysed with cancer histotypes as measured outcome and (epi)genotype as exposure...
September 2016: Journal of Pediatrics
Jia Hu, L U Jin, Tao He, Yifan Li, Yang Zhao, Y U Ding, Xianxin Li, Yunchu Liu, Yaoting Gui, Xiangming Mao, Yongqing Lai, Liangchao Ni
Wilms tumor or nephroblastoma is a common kidney malignant tumor in childhood, accounting for ~5% of all pediatric tumors. At present, reports on Wilms' tumor occurring in adults, particularly at ages >30 years, are extremely rare. The majority of the cases of adult Wilms' tumor are closely associated with chemotherapy. Furthermore, in rare cases, Wilms' tumor is characterized by three classic types of cells, namely blastemal, stromal and epithelial cells. We herein report a case of Wilms' tumor with three classic types of cells on histological examination in a 51 year-old male patient who had received prior chemotherapy...
June 2016: Molecular and Clinical Oncology
Sounak Gupta, Rafael E Jimenez, Andrew L Folpe, John C Cheville
Primary renal leiomyomas and leiomyosarcomas are rare, and there is a paucity of data regarding the pathologic features and outcomes of patients with these tumors. The objective of this study was to review a large series of renal smooth muscle tumors, in order to more fully elucidate their natural histories. Fifty-seven renal smooth muscle tumors were reviewed for various histopathologic features, and leiomyosarcomas were graded using the French Federation of Cancer Centers (FNCLCC) system. Tumor cores in tissue microarrays were evaluated for smooth muscle actin, desmin, h-caldesmon, calponin, myogenin, cytokeratin (OSCAR), CD117, Ki67, estrogen receptor (ER), progesterone receptor (PR), and Wilms tumor 1 (WT1)...
June 7, 2016: American Journal of Surgical Pathology
Eugene B Cone, Stewart S Dalton, Megan Van Noord, Elizabeth T Tracy, Henry E Rice, Jonathan C Routh
PURPOSE: Wilms tumor is the most common childhood renal malignancy and the fourth most common childhood cancer. Many biomarkers have been studied but there has been no comprehensive summary. We systematically reviewed the literature on biomarkers in Wilms tumor to quantify the prognostic implications of the presence of individual tumor markers. MATERIALS AND METHODS: We searched for English language studies from 1980 to 2015 performed in patients younger than 18 years with Wilms tumor and prognostic data...
May 31, 2016: Journal of Urology
Raajit Rampal, Maria E Figueroa
Wilms tumor 1 (WT1) has long been implicated in acute myeloid leukemia. It has been described to be both overexpressed and mutated in different forms of acute myeloid leukemia, and overexpression has been reported to play a prognostic role in this disease. However, the precise mechanism through which WT1 may play a role in leukemogenesis has remained elusive. In recent years, new evidence has emerged that points towards a novel role of WT1 mutations in the deregulation of epigenetic programs in leukemic cells through its interaction with TET proteins...
June 2016: Haematologica
Aurélien Scalabre, Christophe Bergeron, Frederic Brioude, Linda Dainese, Claire Cropet, Aurore Coulomb L'hermine, Claudia Pasqualini, Frederic Auber, Arnauld Verschuur, Gudrun Schleiermacher, Yves Le Bouc, Georges Audry, Sabine Irtan
BACKGROUND: Patients with Beckwith-Wiedemann syndrome (BWS) or isolated hemihypertrophy (HH) treated for a Wilms tumor (WT) carry an increased risk of developing metachronous lesion. There are no guidelines on precise indications for nephron sparing surgery (NSS) in unilateral WT (UWT). The objective of this retrospective study was to delineate the indications of NSS in patients with BWS/HH treated for WT and to evaluate their outcome. PROCEDURE: All cases of BWS/HH treated for a WT according to SIOP protocols from 1980 to 2013 were reviewed...
September 2016: Pediatric Blood & Cancer
Hua Fan, Qian-Qian Shao, Han-Zhong Li, Yu Xiao, Yu-Shi Zhang
We describe the clinical presentation, diagnosis, treatment, and follow-up data of a 39-year-old woman with asymptomatic right kidney tumor, which was later histopathologically diagnosed as metanephric adenoma (MA). Macroscopically, the tumor had integrity tegument with homogeneous and gray cutting surface. Microscopically, the tumor cells were formed in adenoid or papillary pattern and contained psammoma bodies, without distinctive atypia. Immunohistochemistry results showed they were negative for creatine kinase 7, epithelial membrane antigen, and renal cell carcinoma, and positive for AE1/AE3, vimentin, and Wilms Tumor 1...
May 2016: Medicine (Baltimore)
Bacem A E O Khalele, Rami A Al-Shiaty
This study aims at investigating the pathogenesis and oncogenesis of ameloblastoma. Being the commonest odontogenic tumor with idiopathic nature, ameloblastoma poses a fierce controversy about its oncogenesis. Immunohistochemical markers, over years, have highlighted specific pathways which are inherently undertaken in the tumorigenic process of ameloblastoma. Besides the recently pronounced clue of BRAF V600E mutant gene, this study introduces a new marker with its outstanding impact on our contemporary knowledge about ameloblastoma...
June 2016: Annals of Diagnostic Pathology
Iris A L M van Rooij, Loes F M van der Zanden, Ernie M H F Bongers, Kirsten Y Renkema, Charlotte H W Wijers, Michelle Thonissen, Elisabeth M J Dokter, Carlo L M Marcelis, Ivo de Blaauw, Marc H W A Wijnen, Peter M Hoogerbrugge, Jos P M Bokkerink, Michiel F Schreuder, Linda Koster-Kamphuis, Elisabeth A M Cornelissen, Livia Kapusta, Arno F J van Heijst, Kian D Liem, Robert P E de Gier, Anne Marie Kuijpers-Jagtman, Ronald J C Admiraal, Stefaan J Bergé, Jan Jaap van der Biezen, An Verdonck, Vincent Vander Poorten, Greet Hens, Jasmien Roosenboom, Marc R Lilien, Tom P de Jong, Paul Broens, Rene Wijnen, Alice Brooks, Barbara Franke, Han G Brunner, Carine E L Carels, Nine V A M Knoers, Wout F J Feitz, Nel Roeleveld
BACKGROUND: Research regarding the etiology of birth defects and childhood cancer is essential to develop preventive measures, but often requires large study populations. Therefore, we established the AGORA data- and biobank in the Netherlands. In this study, we describe its rationale, design, and ongoing data collection. METHODS: Children diagnosed with and/or treated for a structural birth defect or childhood cancer and their parents are invited to participate in the AGORA data- and biobank...
August 2016: Birth Defects Research. Part A, Clinical and Molecular Teratology
Shirish S Chandanwale, Tushar Kamble, Neha Mishra, Harsh Kumar, Rahul Jadhav
Primary transitional cell carcinoma (TCC) of the ovary is a rare and recently recognized subtype of ovarian surface epithelial-stromal cancer. Pure forms of the TCC ovary account for only 1% of surface epithelial carcinomas. The clinical presentation is indistinguishable from other types of ovarian cancers. They have a favorable response to chemotherapy than other surface epithelial cancers. We report a case of 55-year-old woman who presented with a hard mass in the abdomen. Computed tomography-diagnosed it as a carcinoma of the ovary...
April 2016: International Journal of Applied and Basic Medical Research
Armando J Lorenzo, Rodrigo L P Romao
OBJECTIVE: This review aims at evaluating the current status of minimally invasive surgery at the difficult crossroad between the attractiveness of innovation faced against the solid outcomes offered by the current gold standard, specifically concerning pediatric and adolescent urologic oncology conditions. METHODS: This is a critical review of the literature and current paradigms on the use of minimally invasive surgery for pediatric and adolescent urologic oncology cancers...
May 2016: Urology
Zhang Xiao, Jia Guifang
N(6)-methyladenosine (m(6)A) is one of the most prevalent internal modifications in eukaryotic messenger RNA. The dynamic and reversible modification is installed by methyltransferase complex charactered three subunits: METTL3 (Methyltransferase-like protein 3), METTL14 (Methyltransferase-like protein 14) and WTAP (Wilms tumor 1-associating protein), and erased by two independent demethylases, FTO (Fat mass and obesity associated protein) and ALKBH5 (AlkB homolog 5), in an α-ketoglutarate (α-KG)- and Fe(II)-dependent manner...
April 2016: Yi Chuan, Hereditas
Ivna Néria Silva Ribamar de Carvalho, Renata Mendes de Freitas, Fernando Regla Vargas
Since their discovery in 2008, cell-free circulating microRNAs have been considered potential biomarkers for various conditions, including pediatric cancer. Diagnosis of pediatric cancer still relies on clinical signs, which sometimes may be non-specific or appear at later stages. Thus, there is a need for a better understanding of molecules that allow a less invasive, early and effective method of cancer diagnosis. Despite the efforts of many researches to set specific miRNAs to be routinely used as diagnostic molecules, no miR has been currently utilized so far...
May 2016: Medical Oncology
Buddhika T B Wijerathne, Robert J Meier, Sujatha S Salgado, Suneth B Agampodi
Kidney diseases are becoming a major cause of global burden with high mortality and morbidity. The origins of most kidney diseases are known, but for some the exact aetiology is not yet understood. Dermatoglyphics is the scientific study of epidermal ridge patterns and it has been used as a non-invasive diagnostic tool to detect or predict different medical conditions that have foetal origin. However, there have been a limited number of studies that have evaluated a dermatoglyphic relationship in different kidney diseases...
2016: SpringerPlus
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