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https://www.readbyqxmd.com/read/27935326/role-of-cd56-in-normal-kidney-development-and-wilms-tumorigenesis
#1
Li-Wei Yap, Jesper Brok, Kathy Pritchard-Jones
The cell-surface glycoprotein CD56 has three major isoforms that play important roles in cell adhesion and signaling, which may promote cell proliferation, differentiation, survival, or migration. It is an important molecule in normal kidney development and acts as a key marker in Wilms tumor stem and progenitor cells. Here, we review the structural and genetic features of the CD56 glycoprotein, and summarize its roles in the normal versus diseased metanephric blastema. We discuss areas of CD56-related research that may complement or improve existing Wilms tumor treatment strategies, including the antibody-drug conjugate lorvotuzumab mertansine that binds to CD56...
December 9, 2016: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/27934559/recurrent-nephrotic-syndrome-after-renal-transplant-in-children
#2
Bassam Saeed, Huda Mazloum
OBJECTIVES: Recurrent disease occurs in around 30% of children transplanted for steroid-resistant nephrotic syndrome. Its precipitating risk factors have rarely been studied in the Middle East. The aim of our study was to determine what characterizes posttransplant recurrence of nephrotic syndrome in Syrian children. MATERIALS AND METHODS: We performed a retrospective analysis of 12 nephrotic children who received 1 renal allograft at the Kidney Hospital in Damascus from 2002 to 2013...
December 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27924074/optimized-t-cell-receptor-mimic-tcrm-chimeric-antigen-receptor-t-cells-directed-towards-the-intracellular-wilms-tumor-1-antigen
#3
S Rafiq, T J Purdon, A F Daniyan, M Koneru, T Dao, C Liu, D A Scheinberg, R J Brentjens
CD19-directed chimeric antigen receptor (CAR) T-cells are clinically effective in a limited set of leukemia patients. However, CAR T-cell therapy thus far has been largely restricted to targeting extracellular tumor-associated antigens (TAA). Herein, we report a T-cell receptor-mimic (TCRm) CAR, termed WT1-28z, that is reactive to a peptide portion of the intracellular onco-protein Wilms Tumor 1(WT1), as it is expressed on the surface of the tumor cell in the context of HLA-A*02:01. T-cells modified to express WT1-28z specifically targeted and lysed HLA-A*02:01+ WT1+ tumors and enhanced survival of mice engrafted with HLA-A*02:01+, WT1+ leukemia or ovarian tumors...
December 7, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/27922671/differential-detection-of-cytoplasmic-wilms-tumor-1-expression-by-immunohistochemistry-western-blotting-and-mrna-quantification
#4
Takehiro Maki, Hiroaki Ikeda, Aki Kuroda, Noriaki Kyogoku, Yoshiyuki Yamamura, Yukiko Tabata, Takehiro Abiko, Takahiro Tsuchikawa, Yasuhiro Hida, Toshiaki Shichinohe, Eiichi Tanaka, Kichizo Kaga, Kanako Hatanaka, Yoshihiro Matsuno, Naoko Imai, Satoshi Hirano
Wilms tumor 1 (WT1) is considered to be a promising target of cancer treatment because it has been reported to be frequently expressed at high levels in various malignancies. Although WT1-targeted cancer treatment has been initiated, conclusive detection methods for WT1 are not established. The present study aimed to consolidate immunohistochemistry for WT1 with statistical basis. Transfected cells with forced WT1 expression yielded specific western blot bands and nuclear immunostaining; cytoplasmic immunostaining was not specifically recognized...
December 2, 2016: International Journal of Oncology
https://www.readbyqxmd.com/read/27911068/-laparoscopic-nephron-sparing-surgery-for-wilms-tumor-description-of-two-cases
#5
L López, M Copete, P Villamizar
Surgical resection is the mainstay of treatment for Wilms tumor. The research progress of the large study groups worldwide has reduced mortality. However, searching to minimize morbidity, the minimally invasive approach has been applied in selected patients. This article describes two cases of Wilms tumor managed with laparoscopic nephron sparing surgery. The case 1 with Beckwith-Wiedemann syndrome and bilateral disease, and the case 2 non-syndromic unilateral. The approach was intraperitoneally, without intraoperative complications...
January 25, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/27907819/giant-primary-malignant-mesothelioma-of-the-liver-a-case-report
#6
Ruba Haji Ali, Mohamad Khalife, Ghina El Nounou, Ruba Zuhri Yafi, Hussein Nassar, Zeinab Aidibe, Randa Raad, Rania Abou Eid, Walid Faraj
INTRODUCTION: Malignant mesothelioma is a rare neoplasm of mesothelial cells arising most frequently in the pleura or peritoneum and less frequently in the liver. CASE PRESENTATION: We present a case of primary hepatic mesothelioma of 41year old woman. She had no history of asbestos exposure or cancer. Abdominal computed tomography (CT) showed 21cm intrahepatic mass in the right lobe with many cystic lesions and few small calcifications. Pathology showed a biphasic cellular pattern...
November 11, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27904661/a-novel-sphingosine-kinase-1-inhibitor-ski-5c-induces-cell-death-of-wilms-tumor-cells-in-vitro-and-in-vivo
#7
Zhi-Heng Li, Yan-Fang Tao, Li-Xiao Xu, He Zhao, Xiao-Lu Li, Fang Fang, Yi Wu, Jun Lu, Yan-Hong Li, Wei-Wei Du, Jun-Li Ren, Yi-Ping Li, Yun-Yun Xu, Xing Feng, Jian Wang, Wei-Qi He, Jian Pan
Sphingosine kinase 1 (SphK1) is over-expressed in many cancers and therefore serves as a biomarker for cancer prognosis. SKI-5C is a new SphK1 inhibitor, and until now its molecular function in Wilms' tumor cells remained unknown. Here, using CCK-8 and nude mice experiments we assessed cell growth in Wilms' tumor cell lines (SK-NEP-1 and G401) in vitro and in vivo. We demonstrated that SphK1 is highly expressed in SK-NEP-1 and G401 cells, and through annexin V/propidium iodide staining and flow cytometry analysis, we detected cell apoptosis...
2016: American Journal of Translational Research
https://www.readbyqxmd.com/read/27900278/metanephric-adenofibroma-masquerading-as-wilms-tumor
#8
Prince Raj, Ashwini Khanolkar, Yogesh Kumar Sarin
Metanephric adenofibroma is a rare, biphasic, benign tumor containing both stromal and epithelial components and could be potentially mistaken as Wilms' tumor (WT). We present a 5-year-old girl who was suspected to have metastatic Wilms' tumor on radiological investigations/tru-cut biopsy and had received neoadjuvant chemotherapy, but postoperatively final histopathology revealed it as metanephric adenofibroma. No postoperative chemotherapy was given.
November 2016: APSP Journal of Case Reports
https://www.readbyqxmd.com/read/27900084/sister-mary-joseph-nodule-caused-by-metastatic-desmoplastic-small-round-cell-tumor-a-clinicopathological-report
#9
Noppadol Larbcharoensub, Atcharaporn Pongtippan, Duangjai Pangpunyakulchai, Sith Phongkitkarun, Panuwat Lertsithichai, Thitiya S Dejthevaporn
Sister Mary Joseph nodule is an uncommon metastatic intra-abdominal malignancy involving the umbilicus. The present study describes a rare case of desmoplastic small round cell tumor (DSRCT), histological grade 3, high grade, Gilly classification 4, stage IV, in an 18-year-old Thai man presenting with the Sister Mary Joseph nodule, ascites and pleural effusion. The histopathological examination of the umbilical mass revealed the presence of malignant small round cells associated with prominent stromal desmoplasia...
November 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27900039/networks-analysis-of-genes-and-micrornas-in-human-wilms-tumors
#10
Jimin He, Xiaoxin Guo, Linlin Sun, Kuhao Wang, Haiying Yao
Wilms' tumor (WT) is a common kidney cancer. To date, the expression of genes [transcription factors (TFs), target genes and host genes] and microRNAs (miRNAs/miRs) in WTs has captured the attention of biologists, while the regulatory association between the genes and miRNAs remains unclear. In the present study, TFs, miRNAs, target genes and host genes were considered as key factors in the construction of three levels of regulatory networks, namely, the differentially-expressed network, the related network and the global network...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27896368/targeting-of-the-wt191-138-fragment-to-human-dendritic-cells-improves-leukemia-specific-t-cell-responses-providing-an-alternative-approach-to-wt1-based-vaccination
#11
Nergui Dagvadorj, Anne Deuretzbacher, Daniela Weisenberger, Elke Baumeister, Johannes Trebing, Isabell Lang, Carolin Köchel, Markus Kapp, Kerstin Kapp, Andreas Beilhack, Thomas Hünig, Hermann Einsele, Harald Wajant, Götz Ulrich Grigoleit
Due to its immunogenicity and overexpression concomitant with leukemia progression, Wilms tumor protein 1 (WT1) is of particular interest for immunotherapy of AML relapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT). So far, WT1-specific T-cell responses have mainly been induced by vaccination with peptides presented by certain HLA alleles. However, this approach is still not widely applicable in clinical practice due to common limitations of HLA restriction. Dendritic cell (DC) vaccines electroporated with mRNA encoding full-length protein have also been tested for generating WT1-derived peptides for presentation to T-cells...
November 28, 2016: Cancer Immunology, Immunotherapy: CII
https://www.readbyqxmd.com/read/27895685/pediatric-peri-operative-fractionated-high-dose-rate-brachytherapy-for-recurrent-wilms-tumor-using-a%C3%A2-reconstructed-freiburg-flap
#12
Emily Flower, Kathy Ngoc Tran, Salman Zanjani, Wayne Smith, Jonathan Karpelowsky, Katie Summerhayes, Edgar Estoesta, Jennifer Chard
PURPOSE: To report peri-operative fractionated high-dose-rate (HDR) brachytherapy with a 3D customized Freiburg flap applicator to treat locally recurrent Wilms' tumor, followed by immediate hyperthermic intraperitoneal chemotherapy for a 16-year-old with a second recurrence of nephroblastoma (Wilms' tumor). MATERIAL AND METHODS: The tumor was excised and surgical bed was treated with fractionated HDR brachytherapy using a Freiburg flap applicator. Hyperthermic intraperitoneal chemotherapy was performed immediately after the removal of brachytherapy applicator...
October 2016: Journal of Contemporary Brachytherapy
https://www.readbyqxmd.com/read/27889611/anti-apoptotic-quinolinate-phosphoribosyltransferase-qprt-is-a-target-gene-of-wilms-tumor-gene-1-wt1-protein-in-leukemic-cells
#13
Tove Ullmark, Giorgia Montano, Linnea Järvstråt, Helena Jernmark Nilsson, Erik Håkansson, Kristina Drott, Björn Nilsson, Karina Vidovic, Urban Gullberg
Wilms' tumor gene 1 (WT1) is a zinc finger transcription factor that has been implicated as an oncogene in leukemia and several other malignancies. When investigating possible gene expression network partners of WT1 in a large acute myeloid leukemia (AML) patient cohort, one of the genes with the highest correlation to WT1 was quinolinate phosphoribosyltransferase (QPRT), a key enzyme in the de novo nicotinamide adenine dinucleotide (NAD+) synthesis pathway. To investigate the possible relationship between WT1 and QPRT, we overexpressed WT1 in hematopoietic progenitor cells and cell lines, resulting in an increase of QPRT expression...
November 23, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/27882234/dysgerminoma-developing-from-an-ectopic-ovary-in-a-patient-with-wagr-syndrome-a-case-report
#14
Rie Miura, Yoshihito Yokoyama, Tatsuhiko Shigeto, Masayuki Futagami, Hideki Mizunuma, Akira Kurose, Kazushi Tsuruga, Shinya Sasaki, Kiminori Terui, Etsuro Ito
WAGR syndrome is caused by an 11p13 deletion and includes Wilms' tumor, aniridia, genitourinary anomalies and mental retardation. We encountered a case of a dysgerminoma originating in an ectopic ovary in a woman with WAGR syndrome. Our patient was a 24-year-old nulliparous woman who was diagnosed with WAGR syndrome. The patient had undergone left nephrectomy for a Wilms' tumor and postoperative chemotherapy at the age of 7 months. She also had a history of glaucoma surgery in both eyes, and was followed up at the Department of Pediatrics for diabetes mellitus, hypertension, liver dysfunction and hyperuricemia...
November 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27878779/total-estimated-effective-doses-from-radiologic-imaging-modalities-of-children-with-cancer-a-single-center-experience
#15
Derya Özyörük, Suna Emir, Hacı Ahmet Demir, Gülşah Bayram Kabaçam, Bahattin Tunç
BACKGROUND: Recently, awareness of the cumulative radiation exposure for pediatric oncology patients has been increasing, together with increased survival rates and longer life expectancy. The aim of our study was to quantify the amount of ionising radiation from imaging modalities of pediatric oncology patients. METHODS: Eighty-eight patients who were diagnosed with childhood cancer and followed up for 5 years between 2004-2014 in our center were included in the study...
November 23, 2016: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/27866185/wilms-tumor-1-wt1-mrna-expression-level-at-diagnosis-is-a-significant-prognostic-marker-in-elderly-patients-with-myelodysplastic-syndrome
#16
Joji Nagasaki, Yasutaka Aoyama, Masayuki Hino, Kentaro Ido, Hiroyoshi Ichihara, Masahiro Manabe, Tadanobu Ohta, Atsuko Mugitani
BACKGROUND/AIMS: A high expression of Wilms tumor 1 (WT1) mRNA occurs in most cases of acute leukemia and myelodysplastic syndrome (MDS). Although there are many reports suggesting that acute myeloid leukemia patients with high expression levels of WT1 mRNA have a relatively poor long-term survival, there are few reports addressing the relationship between WT1 levels and prognosis in MDS. METHODS: We retrospectively analyzed 42 elderly patients with MDS whose WT1 levels at diagnosis were available, and we assessed the relationships between WT1 levels in peripheral blood and preexisting prognostic factors such as World Health Organization prognostic scores and Revised International Prognostic Scoring System risk categories, bone marrow blast percentages, and chromosomal abnormalities linked to a poor prognosis...
November 19, 2016: Acta Haematologica
https://www.readbyqxmd.com/read/27862952/synchronous-occurrence-of-acute-lymphoblastic-leukemia-and-wilms-tumor-in-two-patients-underlying-etiology-and-combined-treatment-plan
#17
Joanna S Yi, Junne Kamihara, Jennifer C Kesselheim, Kimberly Davies, Jack van Hoff, Lewis B Silverman, Elizabeth A Mullen
Synchronous cancers are extraordinarily rare in pediatric patients and present a therapeutic challenge. Patient A presented with synchronous unilateral Wilms tumor (WT) and standard-risk (SR) B-precursor acute lymphoblastic leukemia (ALL). Genetic testing revealed bialleleic BRCA2/FANCD1 mutations. Patient B, after SR B-precursor ALL induction therapy, was noted on fever workup to have a renal mass; pathology demonstrated lesion indeterminate between WT and nephrogenic rest. Therapy was customized for each patient to treat both cancers...
November 15, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27856008/comprehensive-renal-function-evaluation-in-patients-treated-for-synchronous-bilateral-wilms-tumor
#18
Rodrigo B Interiano, M Beth McCarville, Noel Delos Santos, Shenghua Mao, Jianrong Wu, Jeffrey S Dome, Kathleen Kieran, Mark A Williams, Rachel C Brennan, Matthew J Krasin, Daniel M Green, Andrew M Davidoff
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...
October 27, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27855980/editorial-comment-zero-ischemia-laparoscopic-assisted-partial-nephrectomy-for-the-management-of-selected-children-with-wilms-tumor-following-neoadjuvant-chemotherapy
#19
https://www.readbyqxmd.com/read/27845967/what-is-new-in-pediatric-surgical-oncology
#20
Roshni Dasgupta, Deborah Billmire, Jennifer H Aldrink, Rebecka L Meyers
PURPOSE OF REVIEW: As pediatric oncology has become more complex, designing and maintaining pediatric surgical protocols require greater expertise. The primary purpose of this review is to summarize the changes in protocols and new study findings, which have changed surgical practice for children with solid tumors. RECENT FINDINGS: The most common solid tumors treated by surgery are reviewed. There are new methods of surgical techniques such as the use of sentinel lymph node techniques for the assessment of lymph nodes in pediatric sarcoma...
November 14, 2016: Current Opinion in Pediatrics
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