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Wilms tumor

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https://www.readbyqxmd.com/read/28640941/phox2b-reliably-distinguishes-neuroblastoma-among-small-round-blue-cell-tumors
#1
Yin P Hung, John P Lee, Andrew M Bellizzi, Jason L Hornick
AIMS: Neuroblastoma shows considerable histologic overlap with other small round blue cell tumors. PHOX2B, a transcription factor essential for autonomic nervous system development, has been reported as an immunohistochemical marker for neuroblastoma. The purpose of this study was to validate the specificity and diagnostic utility of PHOX2B for peripheral neuroblastic tumors. METHODS AND RESULTS: We evaluated 240 cases (133 in whole-tissue sections; 107 in tissue microarrays), including 76 peripheral neuroblastic tumors [median age 2 years; including 4 adults] and 164 other tumors: 44 Wilms tumors; 20 Ewing sarcomas; 10 each CIC-rearranged round cell sarcomas, poorly differentiated synovial sarcomas, lymphoblastic lymphomas, alveolar rhabdomyosarcomas, embryonal rhabdomyosarcomas, mesenchymal chondrosarcomas, Merkel cell carcinomas, olfactory neuroblastomas, and melanomas; 5 each NUT midline carcinomas and desmoplastic small round cell tumors...
June 22, 2017: Histopathology
https://www.readbyqxmd.com/read/28627003/sonographic-screening-for-wilms-tumor-in-children-with-cloves-syndrome
#2
Caitlin M Peterman, R Dawn Fevurly, Ahmad I Alomari, Cameron C Trenor, Denise M Adams, Sophie Vadeboncoeur, Marilyn G Liang, Arin K Greene, John B Mulliken, Steven J Fishman
BACKGROUND: CLOVES syndrome is associated with somatic mosaic PIK3CA mutations and characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies. Wilms tumor (WT) is a malignant embryonal renal neoplasm associated with hemihypertrophy and certain overgrowth disorders. After identifying WT in a child with CLOVES, we questioned whether ultrasonographic screening was necessary in these patients. METHODS: We retrospectively reviewed patients with CLOVES syndrome in our Vascular Anomalies Center at Boston Children's Hospital between 1998 and 2016 to identify those who developed WT...
June 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28620463/recent-advances-in-the-management-of-wilms-tumor
#3
REVIEW
Roberto I Lopes, Armando Lorenzo
The objective of this article is to present an overview of recent trends in the management of Wilms' tumor. With improved survival rates in the past few decades, critical long-term adverse therapy effects (such as renal insufficiency, secondary malignancies, and heart failure) and prevention measures (i.e. nephron-sparing surgery and minimizing the use of radiotherapy) have gained worldwide attention. Specific disease biomarkers that could help stratify high-risk from low-risk patients, and therefore fine-tune management, are in great demand...
2017: F1000Research
https://www.readbyqxmd.com/read/28616573/yap-and-the-hippo-pathway-in-pediatric-cancer
#4
REVIEW
Atif A Ahmed, Abdalla D Mohamed, Melissa Gener, Weijie Li, Eugenio Taboada
The Hippo pathway is an important signaling pathway that controls cell proliferation and apoptosis. It is evolutionarily conserved in mammals and is stimulated by cell-cell contact, inhibiting cell proliferation in response to increased cell density. During early embryonic development, the Hippo signaling pathway regulates organ development and size, and its functions result in the coordinated balance between proliferation, apoptosis, and differentiation. Its principal effectors, YAP and TAZ, regulate signaling by the embryonic stem cells and determine cell fate and histogenesis...
2017: Molecular & Cellular Oncology
https://www.readbyqxmd.com/read/28612492/encephalocraniocutaneous-lipomatosis-with-wilms-tumor
#5
Çağrı Damar, Ayhan Yaman, Mehmet Ali İkidağ, Esra Pekpak, Asburçe Olgaç
No abstract text is available yet for this article.
June 14, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28601179/pediatric-urinary-system-neoplasms-an-overview-and-update
#6
REVIEW
Michael George, Jeannette M Perez-Rosello, Ali Yikilmaz, Edward Y Lee
Pediatric urinary system neoplasms are a diverse group of tumors that frequently overlap in their clinical and radiologic features. By contrast, the histopathologic classification and treatment of these entities have become increasingly refined, resulting in improved outcomes, with the overall survival of Wilms tumors now exceeding 90%. Significantly, many contemporary protocols rely on radiologic diagnosis in the absence of tissue confirmation. This review article provides up-to-date clinical, epidemiologic, and imaging findings of pediatric urinary system neoplasms and their mimics frequently encountered in daily clinical practice...
July 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28600464/biallelic-mutations-in-trip13-increase-the-risk-of-wilms-tumor
#7
(no author information available yet)
Mutations resulting in loss of TRIP13 expression induce chromosome missegregation and SAC impairment.
June 9, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28599697/paraneoplastic-cushing-syndrome-due-to-wilm-s-tumor
#8
Mahwish Faizan, Jaida Manzoor, Muhammad Saleem, Saadia Anwar, Qaiser Mehmood, Ambreen Hameed, Agha Shabbir Ali
Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28598868/pax6-aniridia-syndrome-clinics-genetics-and-therapeutics
#9
Hyun Taek Lim, Dae Hee Kim, Hyuna Kim
PURPOSE OF REVIEW: Aniridia is a rare and panocular disorder affecting most of the ocular structures which may have significant impact on vision. The purpose of this review is to describe the clinical features, genetics, and therapeutic options for this disease and to provide an update of current knowledge and latest research findings. RECENT FINDINGS: Aside from the ocular features, a variety of associated systemic abnormalities, including hormonal, metabolic, gastrointestinal, genitourinary, and neurologic disorders have been reported in children with aniridia...
June 8, 2017: Current Opinion in Ophthalmology
https://www.readbyqxmd.com/read/28598537/factors-possibly-affecting-prognosis-in-children-with-wilms-tumor-diagnosed-before-24-months-of-age-a-report-from-the-associazione-italiana-ematologia-oncologia-pediatrica-aieop-wilms-tumor-working-group
#10
Paolo D'Angelo, Andrea Di Cataldo, Monica Terenziani, Gianni Bisogno, Paola Collini, Martina Di Martino, Fraia Melchionda, Clara Mosa, Marilina Nantron, Daniela Perotti, Giuseppe Puccio, Annalisa Serra, Serena Catania, Filippo Spreafico
BACKGROUND: Children with Wilms' tumor (WT) aged under 24 months (infants) have a better prognosis than older patients. Our aim was to study the epidemiology of this age group, with focus on the modality of diagnosis, tumor size, and association with malformations/syndromes, seeking to understand if any of these factors might be related to prognosis. PATIENTS AND METHODS: Infants diagnosed with WT between 2003 and February 2010 were evaluated. A query form was used to collect data on the modality of WT diagnosis (symptomatic or incidental), tumor volume, maximum diameter, site, and stage...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28591995/-extrarenal-wilms-tumor-report-of-a-case
#11
L L He, H Y Wu, Y Z Tang
No abstract text is available yet for this article.
June 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28583549/reduction-of-slit-diaphragm-associated-molecules-by-sirolimus-is-it-enough-to-induce-proteinuria
#12
B S Kim, J G Lee, Y Cho, S H Song, K H Huh, M S Kim, Y S Kim
Sirolimus (SRL), a mammalian target of rapamycin inhibitor, is widely used in transplantation, but the mechanisms whereby it induces adverse effects, such as proteinuria and edema, remain unclear. To determine whether isolated SRL induces proteinuria or not, the authors intraperitoneally injected C57BL/6 mice with different doses of SRL (0 mg/[kg·d], 3 mg/[kg·d], 10 mg/[kg·d], or 30 mg/[kg·d]) for 24 days. Urinary albumin excretion was then quantified using a double-sandwich enzyme-linked immunosorbent assay, and serum creatinine levels were measured using a single dry-film chemistry auto-analyzer...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28582410/a-phase-i-study-of-single-agent-perifosine-for-recurrent-or-refractory-pediatric-cns-and-solid-tumors
#13
Oren J Becher, Nathan E Millard, Shakeel Modak, Brian H Kushner, Sofia Haque, Ivan Spasojevic, Tanya M Trippett, Stephen W Gilheeney, Yasmin Khakoo, David C Lyden, Kevin C De Braganca, Jill M Kolesar, Jason T Huse, Kim Kramer, Nai-Kong V Cheung, Ira J Dunkel
The PI3K/Akt/mTOR signaling pathway is aberrantly activated in various pediatric tumors. We conducted a phase I study of the Akt inhibitor perifosine in patients with recurrent/refractory pediatric CNS and solid tumors. This was a standard 3+3 open-label dose-escalation study to assess pharmacokinetics, describe toxicities, and identify the MTD for single-agent perifosine. Five dose levels were investigated, ranging from 25 to 125 mg/m2/day for 28 days per cycle. Twenty-three patients (median age 10 years, range 4-18 years) with CNS tumors (DIPG [n = 3], high-grade glioma [n = 5], medulloblastoma [n = 2], ependymoma [n = 3]), neuroblastoma (n = 8), Wilms tumor (n = 1), and Ewing sarcoma (n = 1) were treated...
2017: PloS One
https://www.readbyqxmd.com/read/28576587/-is-nephron-sparing-surgery-relevant-for-unilateral-wilms-tumors
#14
T Tricard, I Lacreuse, V Louis, A Schneider, Y Chaussy, L Soler, R Moog, H Lang, D Jacqmin, F Becmeur
BACKGROUND: Wilms tumors (WTs) are the most frequent renal tumors in children. Radical nephrectomy (RN) remains the gold-standard surgical treatment for this type of cancer. Excellent results in overall survival (>90%) make it possible to consider nephronic preservation. The objective of this systematic review is to evaluate the relevance of nephron-sparing surgery (NSS) for the treatment of nonsyndromic unilateral Wilms tumor (UWT) in children. METHODS: Articles in English related to "unilateral Wilms tumor, unilateral nephroblastoma, partial nephrectomy, nephron-sparing surgery, renal function" identified in the Medline library were screened and data were extracted to perform a qualitative systematic review...
May 30, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28573960/sensitive-detection-of-rare-antigen-specific-t-cells-directed-against-wilms-tumor-1-by-fluorospot-assay
#15
Tina Danielzik, Michael Koldehoff, Ulrike Buttkereit, Dietrich W Beelen, Peter A Horn, Monika Lindemann
No abstract text is available yet for this article.
June 2, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28567073/quantitative-assessment-of-wilms-tumor-1-expression-by-real-time-quantitative-polymerase-chain-reaction-in-patients-with-acute-myeloblastic-leukemia
#16
Hossein Ayatollahi, Mohammad Hadi Sadeghian, Mahmood Naderi, Amir Hossein Jafarian, Seyyede Fatemeh Shams, Neda Motamedirad, Maryam Sheikhi, Afsane Bahrami, Sepideh Shakeri
BACKGROUND: The Wilms tumor 1 (WT1) gene is originally defined as a tumor suppressor gene and a transcription factor that overexpressed in leukemic cells. It is highly expressed in more than 80% of acute myeloid leukemia (AML) patients, both in bone marrow (BM) and in peripheral blood (PB), and it is used as a powerful and independent marker of minimal residual disease (MRD); we have determined the expression levels of the WT1 by real-time quantitative polymerase chain reaction (RQ-PCR) in PB and BM in 126 newly diagnosed AML patients...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28554756/metanephric-stromal-tumor-with-a-rare-incidence-of-squamous-epithelium-a-case-report-and-a-brief-review-of-the-literature
#17
Jia Wang, Wenjie Jin, Xiangru Wu
Metanephric stromal tumor (MST) of the kidney, a rare benign pediatric neoplasm recognized for less than 20 years, is not widely known. The authors describe a case of MST with rare squamous epithelium in a 14-month-old female. A renal mass was discovered during her fetal period. After her birth, computerized tomography revealed that the mass was localized in the inferior pole of her left kidney. She then underwent nephrectomy. The tumor was an unencapsulated but well-defined mass with a white, solid and firm cut surface and had dimensions of 4cm×3...
March 21, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28553959/biallelic-trip13-mutations-predispose-to-wilms-tumor-and-chromosome-missegregation
#18
Shawn Yost, Bas de Wolf, Sandra Hanks, Anna Zachariou, Chiara Marcozzi, Matthew Clarke, Richarda M de Voer, Banafsheh Etemad, Esther Uijttewaal, Emma Ramsay, Harriet Wylie, Anna Elliott, Susan Picton, Audrey Smith, Sarah Smithson, Sheila Seal, Elise Ruark, Gunnar Houge, Jonathon Pines, Geert J P L Kops, Nazneen Rahman
Through exome sequencing, we identified six individuals with biallelic loss-of-function mutations in TRIP13. All six developed Wilms tumor. Constitutional mosaic aneuploidies, microcephaly, developmental delay and seizures, which are features of mosaic variegated aneuploidy (MVA) syndrome, were more variably present. Through functional studies, we show that TRIP13-mutant patient cells have no detectable TRIP13 and have substantial impairment of the spindle assembly checkpoint (SAC), leading to a high rate of chromosome missegregation...
May 29, 2017: Nature Genetics
https://www.readbyqxmd.com/read/28551672/ctnnb1-mutations-in-ovarian-microcystic-stromal-tumors-identification-of-a-novel-deletion-mutation-and-the-use-of-pyrosequencing-to-identify-reported-point-mutation
#19
Kiyong Na, Eun Kyung Kim, Wonjun Jang, Hyun-Soo Kim
BACKGROUND/AIM: Microcystic stromal tumor (MCST) is a rare stromal tumor of the ovary. In this study, we describe clinicopathological characteristics and results of mutational analyses of the CTNNB1gene in two cases of ovarian MCST and we provide a thorough review of previously published cases alongside our current cases and clarify the clinicopathological characteristics of ovarian MCST. PATIENTS AND METHODS: Patients' age was 33 and 31 years, respectively. One patient presented with fever and low abdominal pain, whereas a pelvic mass was incidentally detected in another patient...
June 2017: Anticancer Research
https://www.readbyqxmd.com/read/28550085/knockdown-of-sodium-calcium-exchanger-1-induces-epithelial-to-mesenchymal-transition-in-kidney-epithelial-cells
#20
Sona Lakshme Balasubramaniam, Anilkumar Gopalakrishnapillai, Nicholas J Petrelli, Sonali P Barwe
Mesenchymal to epithelial transition (MET) and epithelial to mesenchymal transition (EMT) are important processes in kidney development. Failure to undergo MET during development leads to the initiation of Wilms tumor (WT) whereas EMT contributes to the development of renal cell carcinomas (RCC). The role of calcium regulators in governing these processes is becoming evident. We demonstrated earlier that Na+/Ca2+ exchanger 1 (NCX1), a major calcium exporter in renal epithelial cells, regulates epithelial cell motility...
May 26, 2017: Journal of Biological Chemistry
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