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wilms tumor review

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https://www.readbyqxmd.com/read/28534317/wt1-alternative-splicing-role-of-its-isoforms-in-neuroblastoma
#1
REVIEW
Daniela Maria Rasà, Agata Grazia D'Amico, Grazia Maugeri, Sebastiano Cavallaro, Velia D'Agata
Wilms tumor 1 (WT1), a tumor suppressor gene, was originally identified in the homonymous renal neoplasm but is also involved in other cancers. Its function is still unclear, since it acts both as a pro- and an anti-oncogene. At least 14 WT1 transcriptional variants have been described; yet most investigations have focused on a small number of isoforms. We describe their structural features and review the evidence of their involvement in cancer with emphasis on neuroblastoma. In future, full characterization of all WT1 isoforms is expected to identify new molecular tumor markers and/or therapeutic targets...
May 22, 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/28521413/chromosome-t-7-11-p15-p15-translocation-in-acute-myeloid-leukemia-coexisting-with-multilineage-dyspoiesis-and-mutations-in-nras-and-wt1-a-case-report-and-literature-review
#2
Jingke Yang, Xiaodong Lyu, Xinghu Zhu, Xiangguang Meng, Wenli Zuo, Hao Ai, Mei Deng
The chromosomal translocation t(7;11)(p15;p15) and the resulting nucleoporin 98-homeobox A9 (NUP98-HOXA9) gene fusion is rare but recurrent genetic abnormity in acute myeloid leukemia (AML). The present study describes a case of AML plus maturation (-M2) with multilineage dyspoiesis in a 30-year-old male in whom a 46,XY,t(7;11)(p15;p15) karyotype was detected through chromosome analysis. Subsequent molecular and sequencing analysis demonstrated a NUP98-HOXA9 fusion gene with a type I fusion between NUP98 exon 12 and HOXA9 exon 1b, and mutations in neuroblastoma V-Ras oncogene homolog and Wilms tumor 1...
May 2017: Oncology Letters
https://www.readbyqxmd.com/read/28481647/the-predictive-value-of-transforming-growth-factor-%C3%AE-in-wilms-tumor-immunopathogenesis
#3
Marla Karine Amarante, Carlos Eduardo Coral de Oliveira, Carolina Batista Ariza, Alberto Yoichi Sakaguchi, Cintya Mayumi Ishibashi, Maria Angelica Ehara Watanabe
Wilms tumor is the most common kidney malignancy in children, especially in children aged less than 6 years. Although therapeutic approach has reached successful rates, there is still room for improvement. Considering the tumor microenvironment, cytokines represent important elements of interaction and communication between tumor cells, stroma, and immune cells. In this regard, the transforming growth factor beta (TGF-β) family members play significant functions in physiological and pathological conditions, particularly in cancer...
May 8, 2017: International Reviews of Immunology
https://www.readbyqxmd.com/read/28449403/effects-of-malnutrition-on-treatment-related-morbidity-and-survival-of-children-with-cancer-in-nicaragua
#4
Allison K Pribnow, Roberta Ortiz, Luis Fulgencio Báez, Luvy Mendieta, Sandra Luna-Fineman
BACKGROUND: Most children with cancer live in resource-limited countries where malnutrition is often prevalent. We identified the relationship between malnutrition and treatment-related morbidity (TRM), abandonment of therapy, and survival of children with cancer in Nicaragua to better inform targeted nutritional interventions. PROCEDURE: We conducted a retrospective review of patients aged 6 months to 18 years with newly diagnosed acute lymphoblastic leukemia, acute myeloid leukemia (AML), Wilms tumor, Hodgkin lymphoma, or Burkitt lymphoma (BL) who were treated between January 1, 2004, and December 31, 2007 at Children's Hospital Manuel de Jesus Rivera in Managua, Nicaragua...
April 27, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28383760/nephrogenic-rests-in-wilms-tumors-treated-with-preoperative-chemotherapy-the-uk-siop-wilms-tumor-2001-trial-experience
#5
Gordan M Vujanić, John R Apps, Veronica Moroz, Federica Ceroni, Richard D Williams, Neil J Sebire, Kathy Pritchard-Jones
BACKGROUND: Nephrogenic rests (NRs) are abnormally persistent foci of embryonal cells, thought to be the precursor lesion of Wilms tumors (WTs). To date, their presence has not been systematically examined in WTs treated with preoperative chemotherapy. METHODS: A systematic analysis of the data on NRs in WTs treated with preoperative chemotherapy obtained from the UK cohort of the International Society of Pediatric Oncology (SIOP) WT 2001 Trial. The study was based on central pathology review of full sets of slides from pathological specimens, with a median of 28 slides reviewed per case...
April 6, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28272669/teratoid-wilms-tumor-report-of-three-cases-and-review-of-the-literature
#6
Doaa Al Ghamdi, Nasir Bakshi, Mohammed Akhtar
Teratoid Wilms tumor is a rare variant of Wilms tumor composed predominantly of well-differentiated epithelial and/or mesenchymal heterologous elements. Like the classical Wilms tumor, this variant may also occur as a renal mass or may be found in extra renal locations. This tumor may be treated effectively by surgical resection; however, it generally fails to respond to chemotherapy. A review of the literature revealed 30 reported cases of intra renal and 5 reports of extra renal teratoid Wilms tumor. We report our experience with an additional three cases of renal teratoid Wilms tumor adding to the 30 cases previously reported...
February 4, 2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28188050/successful-treatment-with-caspofungin-of-candiduria-in-a-child-with-wilms-tumor-review-of-literature
#7
M S Rezai, A Vaezi, H Fakhim, A Soleimani, H Mohammad Jafari, S Mohseni, H Badali
Symptomatic candiduria often occurs in patients with indwelling bladder catheters or immunocompromised host. Isolation of Candida in urine in high-risk patients should primarily be considered as a marker for candidemia. Hematological and genitourinary malignancies are one of the main risk factors associated with Candida urinary tract infections (CUTI). Fluconazole is a choice for initial treatment of CUTI, but it is fluctuate depending on the patient's condition including renal failure, site of urinary infection and Candida species...
February 7, 2017: Journal de Mycologie Médicale
https://www.readbyqxmd.com/read/28125133/acute-wt1-positive-promyelocytic-leukemia-with-hypogranular-variant-morphology-bcr-3-isoform-of-pml-rar%C3%AE-and-flt3-itd-mutation-a-rare-case-report
#8
Xi Zhang, Cheng Yang, Xiangui Peng, Xinghua Chen, Yimei Feng
CONTEXT: Acute promyelocytic leukemia (APL) accounts for 8% to 10% of cases of acute myeloid leukemia (AML). Remission in cases of high-risk APL is still difficult to achieve, and relapses occur readily. CASE REPORT: Here, we describe a case of APL with high white blood cell counts in blood tests and hypogranular variant morphology in bone marrow, together with fms-like tyrosine kinase-3 with internal tandem duplication mutations (FLT3-ITD), and bcr-3 isoform of PML-RARα...
March 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28097788/outcome-of-patients-with-intracranial-relapse-enrolled-on-national-wilms-tumor-study-group-clinical-trials
#9
Rajkumar Venkatramani, Yueh-Yun Chi, Max J Coppes, Marcio Malogolowkin, John A Kalapurakal, Jing Tian, Jeffrey S Dome
BACKGROUND: The occurrence of brain metastases (at diagnosis or at relapse) in patients with Wilms tumor is very rare. METHODS: We retrospectively reviewed the clinical characteristics of patients with Wilms tumor and relapse to the brain enrolled on the National Wilms Tumor Studies (NWTSs) 1-5. RESULTS: Intracranial relapse was documented in 47 patients (0.5%). Of the 45 patients with adequate data, 26 (58%) patients were male. Thirty-eight (84%) patients had favorable histology Wilms tumor...
January 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28097784/reliability-of-intraoperative-frozen-section-for-the-diagnosis-of-renal-tumors-suspicious-for-malignancy-in-children-and-adolescents
#10
Alonso Carrasco, Brian T Caldwell, Carrye R Cost, Brian S Greffe, Timothy P Garrington, Jennifer L Bruny, Jennifer O Black, Nicholas G Cost
BACKGROUND: The ability of intraoperative frozen section (IFS) to reliably diagnose renal tumors in children and adolescents is largely unknown. The objective of our study is to evaluate the ability of IFS to establish a histologic diagnosis for renal tumors in this population. METHODS: We reviewed our experience with patients who underwent IFS at the time of surgery for a renal tumor suspicious for malignancy from 2005 to 2015. The IFS was compared to the final pathology (FP)...
January 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28070651/inferior-vena-cava-involvement-in-children-with-wilms-tumor
#11
Abeer Al Diab, Nader Hirmas, Abdellatif Almousa, Ramiz Abu-Hijlih, Fatinah Aljlouni, Iyad Sultan, Khalil Ghandour
OBJECTIVES: Retrospective review of children with WT thrombus involving the IVC. METHODS: We reviewed the charts of 123 patients with WT diagnosed between January 2006 and December 2015. Patients with caval tumor thrombus were identified, demographic data, radiological images, extent of thrombus, chemo- and radiotherapy, surgical approach, pathology reports and outcomes were analyzed. RESULTS: IVC involvement was identified in 11 patients (9%)...
May 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28018955/wilms-tumor-of-the-ovary
#12
Vinita M Alexander, Jane Meisel, Shannon O'Brien, Namita Khanna
Extrarenal Wilms' tumor of the ovary is a very rare tumor likely derived from embryonic mesonephros. We present the first reported case of a teratoid extrarenal Wilms' tumor of the ovary with a short review of the existing literature. In the case, a 26-year-old woman presented with back pain and was found to have a dermoid cyst; three years later, she presented again, now pregnant, with severe abdominal pain. She was diagnosed with an immature teratoma consisting of a Wilms' tumor (immature component) arising within a mature teratoma and treated exclusively with surgery and surveillance...
February 2017: Gynecologic Oncology Reports
https://www.readbyqxmd.com/read/27993090/wilms-tumor-1-wt1-targeted-cancer-vaccines-to-extend-survival-for-patients-with-pancreatic-cancer
#13
Shigeo Koido, Masato Okamoto, Shigetaka Shimodaira, Haruo Sugiyama
Despite novel chemotherapy treatments, pancreatic ductal adenocarcinoma (PDA) remains a lethal disease. New targeted cancer vaccines may represent a viable option for patients with PDA. The Wilms' tumor 1 (WT1) antigen is one of the most widely expressed tumor-associated antigens in various types of tumors, including PDA. Recent reports have indicated that WT1-targeted cancer vaccines for patients with PDA mediated a potent antitumor effect when combined with chemotherapy in preclinical and clinical studies...
November 2016: Immunotherapy
https://www.readbyqxmd.com/read/27935326/role-of-cd56-in-normal-kidney-development-and-wilms-tumorigenesis
#14
REVIEW
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...
February 2017: Fetal and Pediatric Pathology
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/27856008/comprehensive-renal-function-evaluation-in-patients-treated-for-synchronous-bilateral-wilms-tumor
#16
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...
January 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27845967/what-is-new-in-pediatric-surgical-oncology
#17
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
https://www.readbyqxmd.com/read/27820132/renal-tumors-in-children-younger-than-12-months-of-age-a-65-year-single-institution-review
#18
Margaret G Lamb, Jennifer H Aldrink, Sarah H O'Brien, Han Yin, Michael A Arnold, Mark A Ranalli
Wilms tumor (WT) is the most prevalent pediatric renal tumor and most commonly occurs between ages 1 and 5 years. Data are lacking on children younger than 12 months with renal tumors. The cancer registry at the authors' institution was queried to identify patients 12 months and younger with renal masses. Demographics, clinical presentation, histopathology, stage, and survival outcomes were reviewed. The most common presenting symptoms included an asymptomatic abdominal mass (73%) and hematuria (9%). Histopathology revealed WT in 73% of patients, mesoblastic nephroma in 20%...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27811504/clinical-outcome-and-biological-predictors-of-relapse-after-nephrectomy-only-for-very-low-risk-wilms-tumor-a-report-from-children-s-oncology-group-aren0532
#19
Conrad V Fernandez, Elizabeth J Perlman, Elizabeth A Mullen, Yueh-Yun Chi, Thomas E Hamilton, Kenneth W Gow, Fernando A Ferrer, Douglas C Barnhart, Peter F Ehrlich, Geetika Khanna, John A Kalapurakal, Tina Bocking, Vicky Huff, Jing Tian, James I Geller, Paul E Grundy, James R Anderson, Jeffrey S Dome, Robert C Shamberger
OBJECTIVE: To determine if observation alone after nephrectomy in very low-risk Wilms tumor (defined as stage I favorable histology Wilms tumors with nephrectomy weight <550g and age at diagnosis <2 years) results in satisfactory event-free survival and overall survival, and to correlate relapse with biomarkers. PATIENTS AND METHODS: The AREN0532 study enrolled patients with very low-risk Wilms tumor confirmed by central review of pathology, diagnostic imaging, and surgical reports...
April 2017: Annals of Surgery
https://www.readbyqxmd.com/read/27807337/-giant-solitary-fibrous-tumor-of-the-pleura-a-case-report-and-literature-review
#20
Guangyan Xu, Jianyong Zhang
Solitary fibrous tumor (SFT) is a derived mesenchymal tumor from spindle cells, mostly occurred in the pleura. To analyze the clinical features of the SFT, data for a patient with SFT that involved in the pleura were retrospectively analyzed by assisted thoracoscope in the Affiliated Hospital of Zunyi Medical College in August 2015. The male patient was 45 years old, who showed the main clinical symptoms of chest pain, cough, sputum, and dyspnea. Large amount of right pleural effusion, chest space-occupying lesions were found by chest CT, suggesting a malignant tumor with metastasis at the 2nd and 3rd right rib...
October 28, 2016: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
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