13,710 results match your criteria Wilms Tumor

The Role of Wilms' Tumor Gene (WT1) Expression as a Marker of Minimal Residual Disease in Acute Myeloid Leukemia.

J Clin Med 2022 Jun 9;11(12). Epub 2022 Jun 9.

Division of Hematology and Stem Cell Transplantation, ASUFC, University of Udine, 33100 Udine, Italy.

The Minimal Residual Disease(MRD) monitoring in acute myeloid leukemia (AML) is crucial to guide treatment after morphologic complete remission, to define the need for consolidation with allogeneic stem cell transplantation (Allo-SCT), and to detect impending relapse allowing early intervention. However, more than 50% of patients with AML lack a specific or measurable molecular marker to monitor MRD. We reviewed the key studies on WT1 overexpression as a marker of MRD in AML patients undergoing an intensive chemotherapy program, including Allo-SCT. Read More

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Non-Wilms' renal tumors in children: experience with 139 cases treated at a single center.

BMC Urol 2022 Jun 22;22(1):89. Epub 2022 Jun 22.

Department of Urology, National Children's Medical Center, Beijing Children's Hospital of Capital Medical University, No. 56 Nanlishi St, Xicheng District, Beijing, 100045, China.

Background: Pediatric non-Wilms renal tumors (NWRTs), which comprise a small proportion of renal tumors, are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to treatment. We performed this study to determine the clinical characteristics, management and prognosis of children with Pediatric NWRTs.

Methods: Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008. Read More

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Congenital Teratocarcinosarcoma With Gene Mutation Presenting as an Ocular Mass.

Pediatr Dev Pathol 2022 Jun 22:10935266221111127. Epub 2022 Jun 22.

609162Al Qassimi Women's and Children's Hospital, Sharjah, United Arab Emirates.

Teratocarcinosarcoma is an extremely rare malignancy of the nasal cavity and paranasal sinuses. It exhibits both sarcomatous and carcinomatous components. Less than 100 cases are reported. Read More

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Finding the way to Wilms tumor by comparing the primary and relapse tumor samples.

Cell Rep Med 2022 Jun;3(6):100667

Molecular Bases of Genetic Risk and Genetic Testing Unit, Department of Research, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.

In this issue of Cell Reports Medicine, Gadd and colleagues presented on behalf of the Children's Oncology Group their comprehensive analysis of genetic changes associated with relapse in children with favorable histology Wilms tumor. Read More

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Diffuse Anaplastic Wilms Tumor in a Child With LAMA2-related Muscular Dystrophy.

J Pediatr Hematol Oncol 2022 Jun 23. Epub 2022 Jun 23.

Division of Hematology-Oncology, Cancer and Blood Disease Institute.

Laminin alpha-2-related muscular dystrophy (LAMA2-MD), caused by mutations in the LAMA2 gene, is inherited in an autosomal recessive manner. There is no known association of LAMA2-MD with cancer predisposition. We present a 4-year-old female with LAMA2-MD and Children's Oncology Group stage III diffuse anaplastic Wilms tumor (DAWT). Read More

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Clinical features and next-generation sequencing landscape of essential thrombocythemia, prefibrotic primary myelofibrosis, and overt fibrotic primary myelofibrosis: a Chinese monocentric retrospective study.

J Cancer Res Clin Oncol 2022 Jun 22. Epub 2022 Jun 22.

Department of Hematology, The Fourth Affiliated Hospital of Zhejiang University School of Medicine, N1 Shangcheng Road, Yiwu, Zhejiang, People's Republic of China.

Objective: Since prefibrotic primary myelofibrosis (pre-PMF) was recognized as a separate entity in the 2016 revised classification of MPN differed from essential thrombocythemia (ET) or overt fibrotic primary myelofibrosis (overt PMF), it has been a subject of debate among experts due to its indefinite diagnosis.

Methods: We retrospectively reviewed the clinical parameters, haematologic information, and genetic mutations of patients who were diagnosed with myeloproliferative neoplasms (MPNs) according to the WHO 2016 criteria in China, including 56 ET patients, 19 pre-PMF patients, and 43 overt PMF patients.

Results: Pre-PMF patients exhibited higher leukocyte counts [14. Read More

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LncRNA H19 inhibits proliferation and enhances apoptosis of nephroblastoma cells by regulating the miR-675/TGFBI axis.

Eur Rev Med Pharmacol Sci 2022 Jun;26(11):3800-3806

Department of Neonatal Care Unit, Shandong Province Shanxian Central Hospital, Heze, China.

Objective: To investigate the influences of long non-coding ribonucleic acid (lncRNA) H19 on proliferation and apoptosis of nephroblastoma cells.

Materials And Methods: A total of 5 pairs of nephroblastoma tissues and paraneoplastic tissues were obtained. Gene expression levels of lncRNA H19, microRNA (miR)-675, and transforming growth factor beta induced (TGFBI) were detected via quantitative Reverse Transcription-Polymerase Chain Reaction (qRT-PCR). Read More

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WT1-specific TCRs directed against newly identified peptides install antitumor reactivity against acute myeloid leukemia and ovarian carcinoma.

J Immunother Cancer 2022 Jun;10(6)

Department of Hematology, Leiden University Medical Center, Leiden, The Netherlands

Background: Transcription factor Wilms' tumor gene 1 (WT1) is an ideal tumor target based on its expression in a wide range of tumors, low-level expression in normal tissues and promoting role in cancer progression. In clinical trials, WT1 is targeted using peptide-based or dendritic cell-based vaccines and T-cell receptor (TCR)-based therapies. Antitumor reactivities were reported, but T-cell reactivity is hampered by self-tolerance to WT1 and limited number of WT1 peptides, which were thus far selected based on HLA peptide binding algorithms. Read More

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Spontaneous xenogeneic GvHD in Wilms' tumor Patient-Derived xenograft models and potential solutions.

Animal Model Exp Med 2022 Jun 20. Epub 2022 Jun 20.

Department of Applied Cell Sciences, School of Advanced Technologies in Medicine, Tehran University of Medical Sciences, Tehran, Iran.

Severely immunocompromised NOD.Cg-Prkdc Il2rg (NOG) mice are among the ideal animal recipients for generation of human cancer models. Transplantation of human solid tumors having abundant tumor-infiltrating lymphocytes (TILs) can induce xenogeneic graft-versus-host disease (xGvHD) following engraftment and expansion of the TILs inside the animal body. Read More

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Machine Learning Analysis Reveals Biomarkers for the Detection of Neurological Diseases.

Front Mol Neurosci 2022 31;15:889728. Epub 2022 May 31.

Centre for Host-Microbiome Interactions, Faculty of Dentistry, Oral & Craniofacial Sciences, King's College London, London, United Kingdom.

It is critical to identify biomarkers for neurological diseases (NLDs) to accelerate drug discovery for effective treatment of patients of diseases that currently lack such treatments. In this work, we retrieved genotyping and clinical data from 1,223 UK Biobank participants to identify genetic and clinical biomarkers for NLDs, including Alzheimer's disease (AD), Parkinson's disease (PD), motor neuron disease (MND), and myasthenia gravis (MG). Using a machine learning modeling approach with Monte Carlo randomization, we identified a panel of informative diagnostic biomarkers for predicting AD, PD, MND, and MG, including classical liver disease markers such as alanine aminotransferase, alkaline phosphatase, and bilirubin. Read More

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Isolated steroid-resistant nephrotic syndrome in a Chinese child carrying a de novo mutation in WT1 gene:a case report and literature review.

BMC Pediatr 2022 Jun 16;22(1):349. Epub 2022 Jun 16.

Department of Pediatrics, Shunde Women and Children's Hospital of Guangdong Medical University (Maternity and Child Healthcare Hospital of Shunde Foshan), Foshan, Guangdong Province, China.

Background: Isolated steroid-resistant nephrotic syndrome (ISRNS) is caused by mutations in the Wilms' tumor-1 (WT1) gene, which encodes glomerular podocytes and podocyte slit diaphragm.We report a novel 8-year-old female patient with ISRNS carrying a de novo missense mutation in WT1 gene and presenting a new type of pathology, have never been reported.We also systematically review previous reports of ISRNS in Chinese children. Read More

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The DNA methylation landscape of five pediatric-tumor types.

PeerJ 2022 10;10:e13516. Epub 2022 Jun 10.

Department of Biology, Brigham Young University, Provo, Utah, United States.

Fewer DNA mutations have been identified in pediatric tumors than in adult tumors, suggesting that alternative tumorigenic mechanisms, including aberrant DNA methylation, may play a prominent role. In one epigenetic process of regulating gene expression, methyl groups are attached at the 5-carbon of the cytosine ring, leading to 5-methylcytosine (5mC). In somatic cells, 5mC occurs mostly in CpG islands, which are often within promoter regions. Read More

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Dynamic Epicardial Contribution to Cardiac Interstitial c-Kit and Sca1 Cellular Fractions.

Front Cell Dev Biol 2022 30;10:864765. Epub 2022 May 30.

Department of Animal Biology, Faculty of Sciences, University of Málaga, Málaga, Spain.

The cardiac interstitial cellular fraction is composed of multiple cell types. Some of these cells are known to express some well-known stem cell markers such as c-Kit and Sca1, but they are no longer accepted to be true cardiac stem cells. Although their existence in the cardiac interstitium has not been disputed, their dynamic throughout development, specific embryonic origin, and potential heterogeneity remain unknown. Read More

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Review of Perioperative Care Pathway for Children With Renal Tumors.

Cureus 2022 May 11;14(5):e24928. Epub 2022 May 11.

Biostatistics and Epidemiology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, PAK.

Introduction Wilms tumor is the most common (90%) renal tumor in children. With the recent advances survival rate approaches 90%. This study was designed to identify factors associated with early recovery and hospital discharge, a step forward in the future development of early recovery after surgery (ERAS) protocol in children. Read More

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Wilms tumor reveals DNA repair gene hyperexpression is linked to lack of tumor immune infiltration.

J Immunother Cancer 2022 Jun;10(6)

Pathology, University of Chicago Department of Medicine, Chicago, Illinois, USA

Background: A T cell-rich tumor microenvironment has been associated with improved clinical outcome and response to immune checkpoint blockade therapies in several adult cancers. Understanding the mechanisms for lack of immune cell infiltration in tumors is critical for expanding immunotherapy efficacy. To gain new insights into the mechanisms of poor tumor immunogenicity, we turned to pediatric cancers, which are generally unresponsive to checkpoint blockade. Read More

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Case Report: 2-Year-Old With Wilms Tumors, Familial Heterozygous DIS3L2 Mutation, and Cutis Mamorata Telangiectatica Congenita.

J Pediatr Hematol Oncol 2022 Jun 9. Epub 2022 Jun 9.

Department of Genetics and Genome Sciences, Case Western Reserve University.

Biallelic variants in DI3SL2 cause Perlman Syndrome, associated increased risk for Wilms tumor. Cutis Mamorata Telangiectatica Congenita (CMTC) is a rare congenital disorder characterized by cutaneous vascular anomalies. We report a 2-year-old boy with both Wilms tumor and CMTC. Read More

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Bilateral Gonadoblastoma in a 6-Year Old Girl With Frasier Syndrome: Need for Early Preventive Gonadectomy.

J Pediatr Hematol Oncol 2022 Jun 8. Epub 2022 Jun 8.

Third Department of Pediatrics.

Frasier syndrome (FS) is a rare condition, caused by splice-site mutations of intron 9 in the Wilms' tumor suppressor gene 1 (WT1 gene). The WT1 protein is essential for urogenital development and patients with 46XY karyotype present with female (FS type 1) or male phenotype, gonadal dysgenesis, progressive glomerulopathy, and high risk of gonadoblastoma. We describe a female patient with an IVS9+4C>T donor splice-site mutation, who underwent a preventive gonadectomy at the age of 6 years due to imaging findings of dysplastic gonads. Read More

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An oral WT1 protein vaccine composed of WT1-anchored, genetically engineered Bifidobacterium longum allows for intestinal immunity in mice with acute myeloid leukemia.

Cancer Immunol Immunother 2022 Jun 14. Epub 2022 Jun 14.

Department of Pediatrics, Osaka University Graduate School of Medicine, Suita, Osaka, Japan.

Wilms' tumor 1 (WT1) is a promising tumor-associated antigen for cancer immunotherapy. We developed an oral protein vaccine platform composed of WT1-anchored, genetically engineered Bifidobacterium longum (B. longum) and conducted an in vivo study in mice to examine its anticancer activity. Read More

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Asymptomatic adult Wilms' tumor: A case report.

Radiol Case Rep 2022 Aug 7;17(8):2802-2805. Epub 2022 Jun 7.

Department of Radiology, The 944th Hospital of Joint Logistics Support Force of People's Liberation Army, 22 Xiongguan Road, Jiuquan, 735000, Gansu, China.

Wilms' tumor, also called nephroblastoma, is an extremely uncommon kidney tumor of adulthood. We reported a adult man with a left kidney mass diagnosed as Wilms' tumor. Case presentation: A 25-year-old man was hospitalized due to injury of the anterior cruciate ligament of the right knee. Read More

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Diagnosis, clustering, and immune cell infiltration analysis of m6A-related genes in patients with acute myocardial infarction-a bioinformatics analysis.

J Thorac Dis 2022 May;14(5):1607-1619

Department of Internal Medicine-Cardiovascular Disease, The First Affiliated Hospital of Jinzhou Medical University, Jinzhou, China.

Background: Accurate myocardial infarction (AMI) is one of the leading causes of mortality worldwide. N6-methyladenosine (m6A) modification plays an important role in the development of cardiac remodeling and the cardiomyocyte contractile function. The aim of this study is to analyze the m6A-related molecular biological mechanisms of AMI in terms of accurate diagnosis and prognosis. Read More

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[Scutellarin alleviates lipopolysaccharide-induced renal injury via mediating cysteine-rich protein 61-connective tissue growth factor-nephroblastoma overexpressed gene 1 expression to inhibit nuclear factor-κB signaling pathway].

Zhonghua Wei Zhong Bing Ji Jiu Yi Xue 2022 Apr;34(4):400-406

Department of Nephrology, the Affiliated Hospital of Qingdao University, Qingdao 266003, Shandong, China.

Objective: To explore the protective effect and mechanism of scutellarin (Scu) on sepsis associated-acute kidney injury (SA-AKI).

Methods: (1) In vivo experiment: 36 male C57BL/6 mice were divided into normal saline (NS) control group, lipopolysaccharide (LPS) induced SA-AKI model group (LPS group), 20 mg/kg Scu control group (Scu 20 control group), and 5, 10, 20 mg/kg Scu pretreatment groups by random number table with 6 mice in each group. The SA-AKI model was reproduced by intraperitoneal injection of 10 mg/kg LPS. Read More

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The immune landscape of solid pediatric tumors.

J Exp Clin Cancer Res 2022 Jun 11;41(1):199. Epub 2022 Jun 11.

College of Health and Life Sciences, Hamad Bin Khalifa University, Doha, Qatar.

Background: Large immunogenomic analyses have demonstrated the prognostic role of the functional orientation of the tumor microenvironment in adult solid tumors, this variable has been poorly explored in the pediatric counterpart.

Methods: We performed a systematic analysis of public RNAseq data (TARGET) for five pediatric tumor types (408 patients): Wilms tumor (WLM), neuroblastoma (NBL), osteosarcoma (OS), clear cell sarcoma of the kidney (CCSK) and rhabdoid tumor of the kidney (RT). We assessed the performance of the Immunologic Constant of Rejection (ICR), which captures an active Th1/cytotoxic response. Read More

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Genomic landscape of T-cell lymphoblastic lymphoma.

Chin J Cancer Res 2022 Apr;34(2):83-94

Department of Oncology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.

Objective: T-cell lymphoblastic lymphoma (T-LBL) is an aggressive neoplasm of precursor T cells, however, detailed genome-wide sequencing of large T-LBL cohorts has not been performed due to its rarity. The purpose of this study was to identify putative driver genes in T-LBL.

Methods: To gain insight into the genetic mechanisms of T-LBL development, we performed whole-exome sequencing on 41 paired tumor-normal DNA samples from patients with T-LBL. Read More

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Thirty Years' History since the Discovery of Pax6: From Central Nervous System Development to Neurodevelopmental Disorders.

Int J Mol Sci 2022 May 30;23(11). Epub 2022 May 30.

Department of Developmental Neuroscience, Tohoku University Graduate School of Medicine, Sendai 980-8575, Japan.

Pax6 is a sequence-specific DNA binding transcription factor that positively and negatively regulates transcription and is expressed in multiple cell types in the developing and adult central nervous system (CNS). As indicated by the morphological and functional abnormalities in spontaneous mutant rodents, Pax6 plays pivotal roles in various biological processes in the CNS. At the initial stage of CNS development, Pax6 is responsible for brain patterning along the anteroposterior and dorsoventral axes of the telencephalon. Read More

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Machine Learning-Based CT Radiomics Method for Identifying the Stage of Wilms Tumor in Children.

Front Pediatr 2022 23;10:873035. Epub 2022 May 23.

The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.

Purpose: To develop and validate a machine learning-based CT radiomics method for preoperatively predicting the stages (stage I and non-stage I) of Wilms tumor (WT) in pediatric patients.

Methods: A total of 118 patients with WT, who underwent contrast-enhanced computed tomography (CT) scans in our center between 2014 and 2021, were studied retrospectively and divided into two groups: stage I and non-stage I disease. Patients were randomly divided into training cohorts ( = 94) and test cohorts ( = 24). Read More

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Prediction of histopathological local staging by radiological findings and differential diagnosis overview in children with nephroblastoma.

Rom J Morphol Embryol 2021 Oct-Dec;62(4):939-949

Department of Pediatric Surgery, Grigore Alexandrescu Clinical Emergency Hospital for Children, Bucharest, Romania;

Background: Nephroblastoma is the most common renal malignancy in children kidney. They are highly heterogeneous tumors with challenging imagistic and histopathological (HP) differential diagnosis. Imaging is critical for understanding local anatomy, staging and for planning surgical approach. Read More

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Wnt Signaling Interactor WTIP (Wilms Tumor Interacting Protein) Underlies Novel Mechanism for Cardiac Hypertrophy.

Circ Genom Precis Med 2022 Jun 7:101161CIRCGEN121003563. Epub 2022 Jun 7.

Department of Genetics, Stanford University, CA. (H.N.D.J., R.C., M.S., E.A.A.).

Background: The study of hypertrophic cardiomyopathy (HCM)-a severe Mendelian disease-can yield insight into the mechanisms underlying the complex trait of cardiac hypertrophy. To date, most genetic variants associated with HCM have been found in sarcomeric genes. Here, we describe a novel HCM-associated variant in the noncanonical Wnt signaling interactor (Wilms tumor interacting protein) and provide evidence of a role for WTIP in complex disease. Read More

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[National protocol for diagnosis and care of congenital aniridia: Summary for the attending physician].

J Fr Ophtalmol 2022 Jun;45(6):647-652

Inserm 1138, T17, université de Paris, Paris, France; Laboratoire de génétique moléculaire, centre hospitalier universitaire Necker-Enfants Malades, AP-HP, université de Paris, Paris, France.

Congenital aniridia is a rare panocular disease defined by a national diagnostic and care protocol (PNDS) validated by the HAS. In most cases, it is due to an abnormality in the PAX6 gene, located at 11p13. Aniridia is a potentially blinding autosomal dominant disease with high penetrance. Read More

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Generation of a human iPSC line (MPIi008-A) from a patient with Denys-Drash syndrome.

Stem Cell Res 2022 Jul 30;62:102826. Epub 2022 May 30.

Department of Cell and Developmental Biology, Max Planck Institute for Molecular Biomedicine, Röntgenstrasse 20, Münster 48149, Germany; Department of Medical Life Sciences, College of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul, Republic of Korea. Electronic address:

An induced pluripotent stem cell (hiPSC) line (MPIi008-A) was generated from fibroblasts of a 1-year-old male patient with Denys-Drash syndrome using lentiviral delivery of reprogramming factors OCT4, SOX2, KLF4 and c-MYC. The MPIi008-A iPSC line exhibited typical iPSC morphology and normal karyotype, expressed pluripotent stem cell markers, and showed developmental potential to differentiate into derivatives of all three germ layers in vivo. The hiPSC line harbours a heterozygous missense mutation (R394L) in exon 9 of the WT1 gene. Read More

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