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https://www.readbyqxmd.com/read/28731043/gastroblastoma-harbors-a-recurrent-somatic-malat1-gli1-fusion-gene
#1
Rondell P Graham, Asha A Nair, Jaime I Davila, Long Jin, Jin Jen, William R Sukov, Tsung-Teh Wu, Henry D Appelman, Jorge Torres-Mora, Kyle D Perry, Lizhi Zhang, Sara M Kloft-Nelson, Ryan A Knudson, Patricia T Greipp, Andrew L Folpe
Gastroblastoma is a rare distinctive biphasic tumor of the stomach. The molecular biology of gastroblastoma has not been studied, and no affirmative diagnostic markers have been developed. We retrieved two gastroblastomas from the consultation practices of the authors and performed transcriptome sequencing on formalin-fixed paraffin-embedded tissue. Recurrent predicted fusion genes were validated at genomic and RNA levels. The presence of the fusion gene was confirmed on two additional paraffin-embedded cases of gastroblastoma...
July 21, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28729402/wilms-tumor-ncam-expressing-cancer-stem-cells-as-potential-therapeutic-target-for-polymeric-nanomedicine
#2
Ela Markovsky, Einav Vax, Dikla Ben-Shushan, Anat Eldar-Boock, Rachel Shukrun, Eilam Yeini, Iris Barshack, Revital Caspi, Orit Harari-Steinberg, Naomi Pode-Shakked, Benjamin Dekel, Ronit Satchi-Fainaro
Cancer stem cells (CSC) form a specific population within the tumor that has been shown to have self-renewal and differentiation properties, increased ability to migrate and form metastases, and increased resistance to chemotherapy. Consequently, even a small number of cells remaining after therapy can repopulate the tumor and cause recurrence of the disease. CSCs in Wilms tumor, a pediatric renal cancer, were previously shown to be characterized by neural cell adhesion molecule (NCAM) expression. Therefore, NCAM provides a specific biomarker through which the CSC population in this tumor can be targeted...
July 20, 2017: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/28720077/hemolytic-uremic-syndrome-as-the-presenting-manifestation-of-wt1-mutation-and-denys-drash-syndrome-a-case-report
#3
Joseph L Alge, Scott E Wenderfer, John Hicks, Mir Reza Bekheirnia, Deborah A Schady, Jamey S Kain, Michael C Braun
BACKGROUND: Hemolytic uremic syndrome (HUS) can occur as a primary process due to mutations in complement genes or secondary to another underlying disease. HUS sometimes occurs in the setting of glomerular diseases, and it has been described in association with Denys-Drash syndrome (DDS), which is characterized by the triad of abnormal genitourinary development; a pathognomonic glomerulopathy, diffuse mesangial sclerosis; and the development of Wilms tumor. CASE PRESENTATION: We report the case of a 46, XX female infant who presented with HUS and biopsy-proven thrombotic microangiopathy...
July 18, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28720068/inhibitory-effect-of-par-4-combined-with-cisplatin-on-human-wilms-tumor-cells
#4
Jun Wang, Yunjie Li, Fangfang Ma, Huifeng Zhou, Rong Ding, Binbin Lu, Li Zou, Junxia Li, Rugang Lu
Wilms' tumor is associated with a high treatment success rate, but there is still a risk of recurrence. Cisplatin, which is one of the chemotherapeutic agents used for its treatment, is associated with a very high rate of resistance. Par-4 (prostate apoptosis response 4) is a tumor suppressor, which is capable of sensitizing tumor cells to chemotherapy. Therefore, the aim of this study was to determine whether combined treatment with Par-4 and cisplatin is effective for inhibiting growth of Wilms' tumor. Wilms' tumor and control cell samples were collected and analyzed by immunofluorescence assay and immunohistochemistry...
July 2017: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/28719912/crocin-protects-podocytes-against-oxidative-stress-and-inflammation-induced-by-high-glucose-through-inhibition-of-nf-%C3%AE%C2%BAb
#5
Sutong Li, Xiaoxia Liu, Jie Lei, Junle Yang, Puxun Tian, Yi Gao
BACKGROUND/AIMS: Diabetic nephropathy (DN) is a microangiopathic disease characterized by excessive urinary albumin excretion, which occurs in 30% of patients with diabetes mellitus. It is the second leading cause of end-stage renal diseases in China. Nuclear factor-kappa B (NF-κB) is reported to be closely correlated with the inflammation underlying diabetes-associated renal damage. Crocin, a plant-derived compound, has antioxidant properties that may inhibit NF-κB. METHODS: In the present study, we used a conditionally immortalized mouse podocyte cell line to explore whether crocin could effectively block albuminuria...
July 18, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28715709/parental-age-and-childhood-cancer-risk-a-danish-population-based-registry-study
#6
Zuelma A Contreras, Johnni Hansen, Beate Ritz, Jorn Olsen, Fei Yu, Julia E Heck
BACKGROUND: Though the association between parental age at child's birth and the risk of childhood cancer has been previously investigated, the evidence to date is inconclusive and scarce for rarer cancer types. METHODS: Cancer cases (N=5,856) were selected from all children born from 1968 to 2014 and diagnosed from 1968 to 2015 in Denmark at less than 16 years of age listed in the nationwide Danish Cancer Registry. Cases were individually matched to controls (1:100) on sex and year of birth with a total of 585,594 controls randomly sampled from all live births in Denmark from the Danish Central Population Registry...
July 14, 2017: Cancer Epidemiology
https://www.readbyqxmd.com/read/28711961/heat-shock-protein-70-nitric-oxide-effect-on-stretched-tubular-epithelial-cells-linked-to-wt-1-cytoprotection-during-neonatal-obstructive-nephropathy
#7
Luciana Mazzei, Fernando Darío Cuello-Carrión, Neil Docherty, Walter Manucha
BACKGROUND: Mechanical stress is a key pathogenic driver of apoptosis in the tubular epithelium in obstructive nephropathy. Heat shock protein 70 (Hsp70) and Wilms' tumor (WT-1) have been proposed to represent linked downstream effectors of the cytoprotective properties of NO. In the present study, we sought to evaluate whether the cytoprotective effects of L-arginine in neonatal obstructive nephropathy may be associated with NO-dependent increases in WT-1 and Hsp70 expression. METHODS: Neonatal Wistar-Kyoto rats were submitted to complete unilateral ureteral obstruction (UUO) and treated thereafter with vehicle, L-NAME or L-arginine by daily gavage for 14 days to block or augment NO levels, respectively...
July 15, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28697171/variability-in-imaging-practices-and-comparative-cumulative-effective-dose-for-neuroblastoma-and-nephroblastoma-patients-at-6-pediatric-oncology-centers
#8
Baptiste Morel, Anne C Jaudeau-Collart, Maia Proisy, Louis M Leiber, Valentin Tissot, Marie P Quéré, Martine Mergy, Isabelle Pellier, Clara Vallin, Dominique Sirinelli
The purpose of this study was to estimate the cumulative effective dose (CED) from diagnosis and posttherapy computed tomographic (CT) scans performed on children treated for neuroblastoma or nephroblastoma (Wilms tumor) and to examine the different imaging practices used in 6 regional pediatric oncology centers between January 2010 and December 2013. We analyzed retrospectively the CT scan acquisition data in children aged 10 years or younger at diagnosis. The use of nonionizing imaging modalities was reported...
July 10, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28695795/pharmacologic-inhibition-of-%C3%A3-catenin-with-pyrvinium-inhibits-murine-and-human-models-of-wilms-tumor
#9
Dina Polosukhina, Harold Love, Harold Moses, Ethan Lee, Roy Zent, Peter Clark
Wilms tumor (WT) is the most common renal malignancy of childhood and the fourth most common pediatric solid malignancyin the United States. While the mechanisms underlying the WT biology are complex, these tumors most often demonstrate activation of the canonical Wnt/ß-catenin pathway. We and others have shown that constitutive activation of ß-catenin restricted to the renal epithelium is sufficient to induce primitive renal epithelial tumors which resemble human WT. Here we demonstrate that pharmacologic inhibition of ß-catenin gene transcription with pyrvinium inhibits tumor growth and metastatic progression in a murine model of WT...
July 10, 2017: Oncology Research
https://www.readbyqxmd.com/read/28693222/identification-of-differentially-expressed-inflammatory-factors-in-wilms-tumors-and-their-association-with-patient-outcomes
#10
Fei Guo, Junjie Zhang, Lei Wang, Wei Zhao, Jiekai Yu, Shu Zheng, Jiaxiang Wang
The present study aimed to identify differentially expressed inflammatory factors observed in Wilms tumors (WT), and to investigate the association of these factors with clinical stage, pathological type, lymph node metastasis and vascular involvement of WT. Surface-enhanced laser desorption/ionization-time of flight mass spectrometry was performed to screen differentially expressed proteins among WT and normal tissue pairs. Upregulated proteins in WT were separated and purified by solid phase extraction and Tricine SDS-PAGE, respectively...
July 2017: Oncology Letters
https://www.readbyqxmd.com/read/28692553/simultaneous-presentation-of-wilms-tumor-and-immature-ovarian-teratoma-in-beckwith-wiedemann-syndrome
#11
Jason C White, Jinglan Liu, Akash Nahar
The Beckwith-Wiedemann syndrome is a cancer predisposition syndrome characterized by a predilection to embryonal tumor growth, especially Wilms tumor, adrenocortical carcinomas, and hepatoblastomas. Genetic analysis of patients has revealed a link to the imprinted domain of the 11p15.5 chromosome and methylation status of the H19 locus and Igf-2. These genes have also been studied in other cancers, including ovarian teratomas. Our case is a patient with a simultaneous presentation of a Wilms tumor and immature ovarian teratoma and subsequently diagnosed with Beckwith-Wiedemann syndrome, which has not been previously described...
July 7, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28690152/decreased-expression-of-orexin-1-receptor-in-adult-mice-testes-during-alloxan-induced-diabetes-mellitus-perturbs-testicular-steroidogenesis-and-glucose-homeostasis
#12
Deepanshu Joshi, Debarshi Sarkar, Shio Kumar Singh
Diabetes mellitus (DM) affects male reproductive system and causes infertility. The male reproductive health is largely dependent upon uptake and proper utilization of glucose by testicular cells. Results show involvement of orexin A (OXA) and its receptor (OX1R) in regulation of steroidogenesis and glucose homeostasis in adult mice testis. However, the role of OX1R in regulation of testicular functions during hyperglycemia has not been investigated so far. The present study, therefore, examined the role of OX1R in regulation of steroidogenesis and glucose homeostasis in testis of adult mice during alloxan-induced type 1 DM...
July 6, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28687950/is-pre-operative-chemotherapy-desirable-in-all-patients-of-wilms-tumor
#13
Akash Kumar, Sameer Bakhshi, Sandeep Agarwala
The timing and role of chemotherapy in the management of Wilms' tumor has long been the matter of debate, with different groups showing equally comparable and encouraging results. Over the last decade, however, both the ideol-ogies seem to be converging and the attempt has been to identify groups benefitting with pre-operative chemotherapy, as well as those, where upfront resection should be attempted. In this article authors intend to discuss pros and cons of both the strategies and their applicability in a resource poor setting in developing countries like India...
July 8, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28680821/a-unified-pathogenesis-for-kidney-diseases-including-genetic-diseases-and-cancers-by-the-protein-homeostasis-system-hypothesis
#14
REVIEW
Kyung-Yil Lee
Every cell of an organism is separated and protected by a cell membrane. It is proposed that harmony between intercellular communication and the health of an organism is controlled by a system, designated the protein-homeostasis-system (PHS). Kidneys consist of a variety of types of renal cells, each with its own characteristic cell-receptor interactions and producing characteristic proteins. A functional union of these renal cells can be determined by various renal function tests, and harmonious intercellular communication is essential for the healthy state of the host...
June 2017: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/28679862/mysm1-2a-dub-is-an-epigenetic-regulator-in-human-melanoma-and-contributes-to-tumor-cell-growth
#15
Christina Wilms, Carsten M Kroeger, Adelheid V Hainzl, Ishani Banik, Clara Bruno, Ioanna Krikki, Vida Farsam, Meinhard Wlaschek, Martina V Gatzka
Histone modifying enzymes, such as histone deacetylases (HDACs) and polycomb repressive complex (PRC) components, have been implicated in regulating tumor growth, epithelial-mesenchymal transition, tumor stem cell maintenance, or repression of tumor suppressor genes - and may be promising targets for combination therapies of melanoma and other cancers. According to recent findings, the histone H2A deubiquitinase 2A-DUB/Mysm1 interacts with the p53-axis in hematopoiesis and tissue differentiation in mice, in part by modulating DNA-damage responses in stem cell and progenitor compartments...
June 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28677722/regulation-of-retinoic-acid-synthetic-enzymes-by-wt1-and-hdac-inhibitors-in-293-cells
#16
Yifan Li, Lei Wang, Weipeng Ai, Nianhui He, Lin Zhang, Jihui Du, Yong Wang, Xingjian Mao, Junqi Ren, Dan Xu, Bei Zhou, Rong Li, Liwen Mai
All-trans retinoic acid (atRA), which is mainly generated endogenously via two steps of oxidation from vitamin A (retinol), plays an indispensible role in the development of the kidney and many other organs. Enzymes that catalyze the oxidation of retinol to generate atRA, including aldehyde dehydrogenase 1 family (ALDH1)A1, ALDH1A2 and ALDH1A3, exhibit complex expression patterns at different stages of renal development. However, molecular triggers that control these differential expression levels are poorly understood...
July 3, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28674120/surveillance-recommendations-for-children-with-overgrowth-syndromes-and-predisposition-to-wilms-tumors-and-hepatoblastoma
#17
REVIEW
Jennifer M Kalish, Leslie Doros, Lee J Helman, Raoul C Hennekam, Roland P Kuiper, Saskia M Maas, Eamonn R Maher, Kim E Nichols, Sharon E Plon, Christopher C Porter, Surya Rednam, Kris Ann P Schultz, Lisa J States, Gail E Tomlinson, Kristin Zelley, Todd E Druley
A number of genetic syndromes have been linked to increased risk for Wilms tumor (WT), hepatoblastoma (HB), and other embryonal tumors. Here, we outline these rare syndromes with at least a 1% risk to develop these tumors and recommend uniform tumor screening recommendations for North America. Specifically, for syndromes with increased risk for WT, we recommend renal ultrasounds every 3 months from birth (or the time of diagnosis) through the seventh birthday. For HB, we recommend screening with full abdominal ultrasound and alpha-fetoprotein serum measurements every 3 months from birth (or the time of diagnosis) through the fourth birthday...
July 1, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28668903/laparoscopically-removed-streak-gonad-revealed-gonadoblastoma-in-frasier-syndrome
#18
Kazunori Hashimoto, Y U Horibe, Jiro Ezaki, Toshiyuki Kanno, Nobuko Takahashi, Yoshika Akizawa, Hideo Matsui, Tomoko Yamamoto, Noriyuki Shibata
BACKGROUND: Frasier syndrome (FS) is characterized by gonadal dysgenesis and progressive nephropathy caused by mutation in the Wilm's tumor gene (WT1). We report a case of FS in which diagnosis was based on amenorrhea with nephropathy, and laparoscopically-removed streak gonad which revealed gonadoblastoma. CASE REPORT: At the age of 3 years, the patient developed nephrotic syndrome. This later became steroid-resistant and, by the age of 16 years, had progressed to end-stage renal failure with peritoneal dialysis...
July 2017: Anticancer Research
https://www.readbyqxmd.com/read/28668862/clinicopathological-characteristics-of-metaplastic-papillary-tumor-of-the-fallopian-tube
#19
M I Jang, Ji-Youn Sung, Ji-Ye Kim, Hyun-Soo Kim
Metaplastic papillary tumor (MPT) of the fallopian tube is a very uncommon lesion, displaying papillary growth of bland-appearing cells with abundant, eosinophilic cytoplasm and mucinous metaplasia. It is difficult for pathologists to determine whether to categorize this lesion as a metaplastic proliferative lesion or a true neoplasm. We recently experienced a case of tubal MPT and initiated a comprehensive review of previously published cases with thorough analysis of clinicopathological characteristics. MPT is typically related to pregnancy, but we describe the first case of pregnancy-unrelated, incidentally detected tubal MPT in a 51-year-old woman who underwent surgery for endometrial cancer...
July 2017: Anticancer Research
https://www.readbyqxmd.com/read/28655531/results-of-the-3-rd-associazione-italiana-ematologia-oncologia-pediatrica-aieop-cooperative-protocol-on-wilms-tumor-tw2003-and-related-considerations
#20
Filippo Spreafico, Davide Biasoni, Salvatore Lo Vullo, Lorenza Gandola, Paolo D'Angelo, Monica Terenziani, Maurizio Bianchi, Provenzi Massimo, Paolo Indolfi, Pession Andrea, Nantron Marilina, Andrea Di Cataldo, Carlo Morosi, Daniela Perotti, Serena Catania, Franca Fossati Bellani, Paola Collini
PURPOSE: TW2003, the 3(rd) Italian prospective study on Wilms tumor (WT), aimed to improve survival in patients with stage III-IV tumors, de-escalate therapy for stage I-II non-anaplastic tumors, refine the risk stratification of therapy, and develop a national infrastructure for biobanking and central pathology review. METHODS: TW2003 recruited children ≤18 years old with primary intrarenal tumors. Local physicians chose nephrectomy with or without preoperative chemotherapy as the initial treatment, based on the risk of unsafe and/or incomplete immediate surgery...
June 24, 2017: Journal of Urology
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