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wilms tumor review

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https://www.readbyqxmd.com/read/29751751/primary-synovial-sarcoma-of-the-kidney-a-case-report-of-complete-pathological-response-at-a-lebanese-tertiary-care-center
#1
Alissar El Chediak, Deborah Mukherji, Sally Temraz, Samer Nassif, Sara Sinno, Rami Mahfouz, Ali Shamseddine
BACKGROUND: Primary synovial sarcoma of the kidney is a rare type of soft tissue sarcoma. Its presenting features can resemble those of other renal tumors; rendering its early diagnosis, a dilemma. Several cases of renal synovial sarcoma have been reported in the literature with varying treatment options and outcomes. This article describes a rare case of primary renal synovial sarcoma and reviews all cases in the literature. CASE PRESENTATION: A 26-year-old male presented with flank pain and hematuria...
May 11, 2018: BMC Urology
https://www.readbyqxmd.com/read/29700824/clinical-molecular-genetics-and-therapeutic-aspects-of-syndromic-obesity
#2
REVIEW
E Geets, M E C Meuwissen, W Van Hul
Obesity has become a major health problem worldwide. To date, more than 25 different syndromic forms of obesity are known in which one (monogenic) or multiple (polygenic) genes are involved. This review gives an overview of these forms and focuses more in detail on six syndromes: Prader Willi Syndrome and Prader Willi like phenotype, Bardet Biedl Syndrome, Alström Syndrome, Wilms tumor, Aniridia, Genitourinary malformations and mental Retardation syndrome and 16p11.2 (micro)deletions. Years of research provided plenty of information on the molecular genetics of these disorders and the obesity phenotype leading to a more individualized treatment of the symptoms, however, a lot of questions still remain unanswered...
April 26, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29629978/wilms-tumor-of-the-uterus
#3
Andre Pinto, Marilyn Huang, Rosa P Castillo, Matthew P Schlumbrecht
Wilms tumor (WT) is an uncommon malignant neoplasm that occurs predominantly in the kidney of pediatric patients; its extrarenal counterpart is exceedingly rare. We present the case of an adult female diagnosed with uterine WT. Following hysterectomy due to a uterine mass, histopathologic examination demonstrated a triphasic malignancy composed of epithelial, stromal, and blastemal elements. The characteristic morphologic features, which were supported by immunohistochemical analysis, were diagnostic of WT of the uterus...
April 6, 2018: International Journal of Gynecological Pathology
https://www.readbyqxmd.com/read/29623275/wilms-tumor-protein-1-and-enzymatic-oxidation-of-5-methylcytosine-in-brain-tumors-potential-perspectives
#4
REVIEW
Ashley Ramsawhook, Alexey Ruzov, Beth Coyle
The patterns of 5-methylcytosine (5mC) and its oxidized derivatives, 5-hydroxymethylcytosine, 5-formylcytosine, and 5-carboxylcytosine (5caC) are reportedly altered in a range of cancers. Likewise, Wilms' Tumor protein 1 (WT1), a transcription factor essential for urogenital, epicardium, and kidney development exhibits aberrant expression in multiple tumors. Interestingly, WT1 directly interacts with TET proteins that catalyze the enzymatic oxidation of 5mC and exhibits high affinity for 5caC-containing DNA substrates in vitro ...
2018: Frontiers in Cell and Developmental Biology
https://www.readbyqxmd.com/read/29564632/infections-caused-by-fusarium-species-in-pediatric-cancer-patients-and-review-of-published-literature
#5
Mariana Volpe Arnoni, Claudete Rodrigues Paula, Marcos Ereno Auler, Cirilo Cesar Naozuka Simões, Shirley Nakano, Maria Walderez Szeszs, Márcia de Souza Carvalho Melhem, Virgínia Bodelão Richini Pereira, Hans Garcia Garces, Eduardo Bagagli, Eriques Gonçalves Silva, Melissa Ferreira de Macêdo, Luciana da Silva Ruiz
Fusarium species have emerged as responsible for a broad spectrum of infections, including superficial, locally invasive and disseminated ones, especially in the hospital environment. Since there are few reports of invasive and disseminated fusariosis in children, the aim of this study was to report four cases of nosocomial infection caused by this microorganism in children with cancer hospitalized in a public children's hospital located in Brazil. Two of these patients were female and two were male. All patients presented febrile neutropenia, while three patients had acute lymphocytic leukemia and one patient had Wilms' tumor as underlying disease...
March 21, 2018: Mycopathologia
https://www.readbyqxmd.com/read/29551244/surgical-outcomes-of-patients-with-beckwith-wiedemann-syndrome
#6
Candace C Style, Stephanie M Cruz, Patricio E Lau, Timothy C Lee, David E Wesson, Oluyinka O Olutoye
PURPOSE: The purpose of this study was to evaluate treatment and surgical outcomes of patients of Beckwith-Wiedemann Syndrome (BWS) treated at a tertiary children's hospital. METHODS: A retrospective review of infants evaluated at Texas Children's Hospital for BWS from August 2000 to December 2016 was performed. Data collected included demographic information, clinical presentation, genetic evaluation, fetal imaging, operative treatment, and outcomes. RESULTS: Forty-seven children with a diagnosis of BWS were identified...
February 12, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29551096/review-ontology-and-endocrinology-of-the-reproductive-system-of-bulls-from-fetus-to-maturity
#7
M McGowan, M K Holland, G Boe-Hansen
This review focuses on current understanding of prenatal, prepubertal and post-pubertal development of the male reproductive system of cattle. The critical developmental events occur during the first 3 to 4 months of gestation and the first ~6 to 9 months after birth. The Wilms Tumor-1 and SRY proteins play critical roles in early development and differentiation of the fetal testis, which in turn drives gestational development of the entire male reproductive system. The hypothalamic-pituitary-gonadal axis matures earlier in the bovine fetus than other domestic species with descent of the testes into the scrotum occurring around the 4th month of gestation...
March 19, 2018: Animal: An International Journal of Animal Bioscience
https://www.readbyqxmd.com/read/29528810/a-blastema-predominant-canine-renal-nephroblastoma-with-gingival-metastasis-case-report-and-literature-review
#8
Bo Chen, Wen-Ta Li, Fun-In Wang
Nephroblastomas are uncommon embryonal tumors in dogs. We report herein a blastema-predominant nephroblastoma with gingival metastasis in an 8-y-old Miniature Pinscher dog. Histologically, the mass was composed mainly of blastemal elements with minor epithelial and mesenchymal differentiation. Metastatic masses in the gingiva had histologic and immunohistochemical features similar to those of the primary renal nephroblastoma. Neoplastic cells were extensively positive for both vimentin and PAX8, and scattered positive for cytokeratin...
May 2018: Journal of Veterinary Diagnostic Investigation
https://www.readbyqxmd.com/read/29501377/laparoscopic-nephrectomy-for-wilms-tumor-can-we-expand-on-the-current-siop-criteria
#9
Katherine Burnand, Annie Roberts, Aurore Bouty, Michael Nightingale, Martin Campbell, Yves Heloury
INTRODUCTION: Wilms' tumor now has a good overall prognosis with open radical nephrectomy having been the mainstay of surgical treatment. Recently laparoscopic nephrectomy (LN) has been growing in popularity. The aim of our study was to review our indications and outcomes for laparoscopic resections for Wilms' tumor and compare indications with International Society of Paediatric Oncology (SIOP) criteria for LN. MATERIAL AND METHODS: Patient demographics, preoperative management, surgical data, respect of SIOP criteria, complications, disease outcome, and follow-up were recorded on consecutive children who underwent nephrectomy for Wilms' tumor...
February 13, 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29489735/cancer-management-in-kabuki-syndrome-the-first-case-of-wilms-tumor-and-a-literature-review
#10
Hideto Teranishi, Yuhki Koga, Kentaro Nakashima, Eiji Morihana, Kanako Ishii, Yasunari Sakai, Tomoaki Taguchi, Yoshinao Oda, Noriko Miyake, Naomichi Matsumoto, Shouichi Ohga
A 3-year-old Japanese girl treated for hypoplastic left heart syndrome and Dandy-Walker syndrome was diagnosed with Kabuki syndrome (KS) with a mutation of KMT2D; c.13285C>T:p.Q4429*. Concurrently, macrohematuria portended the diagnosis of Wilms tumor. Postoperative chemotherapy has achieved complete remission despite a prolonged and reduced regimen due to liver dysfunction and convulsions. Cancer predisposition has been suggested for KS due to oncogenic mutations in KMT2D or KDM6A. The first case of nephroblastoma exemplified the treatability of malignancies in KS patients, as shown in the 9 cases reviewed...
February 27, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29471818/adult-wilms-tumor-with-inferior-vena-cava-thrombus-and-distal-deep-vein-thrombosis-a-case-report-and-literature-review
#11
Krzysztof Ratajczyk, Adrian Czekaj, Joanna Rogala, Pawel Kowal
BACKGROUND: Adult Wilms tumor (WT, nephroblastoma) is a rare, but well-described renal neoplasm. Although inferior vena cava tumor thrombosis is present in up to 10% of Wilms tumors in childhood, only few cases of this clinical manifestation in adults have been reported. To the best of our knowledge, this is the first case of adult WT infiltrating into inferior vena cava (IVC) with concomitant distal deep vein thrombosis. CASE PRESENTATION: A 28-year-old male patient with gross hematuria and right flank pain was diagnosed with right kidney tumor penetrating to IVC...
February 23, 2018: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29460221/the-genetics-of-aniridia-simple-things-become-complicated
#12
REVIEW
Anna Wawrocka, Maciej R Krawczynski
Aniridia is a rare, panocular disorder characterized by a variable degree of hypoplasia or the absence of iris tissue associated with additional ocular abnormalities. It is inherited in an autosomal dominant manner, with high penetrance and variable expression even within the same family. In most cases the disease is caused by haploinsufficiency truncating mutations in the PAX6 gene; however, in up to 30% of aniridia patients, disease results from chromosomal rearrangements at the 11p13 region. The aim of this review is to present the clinical and genetic aspects of the disease...
May 2018: Journal of Applied Genetics
https://www.readbyqxmd.com/read/29445571/immunoreactivity-of-wilms-tumor-1-wt1-as-an-additional-evidence-supporting-hemangiomatous-rather-than-inflammatory-origin-in-the-etiopathogenesis-of-angiolymphoid-hyperplasia-with-eosinophilia
#13
Fatma Tokat, Julia S Lehman, Engin Sezer, Emel Dikicioglu Cetin, Umit Ince, Emel Ozturk Durmaz
Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular proliferative disorder mainly located in the periauricular region. The etiopathogenesis of ALHE is unknown, and it is still controversial as to whether the entity represents a benign vascular neoplasm or an inflammatory process. Aim: Recently, the intracytoplasmic staining pattern of Wilms tumor 1 (WT1) on immunohistochemistry has highlighted true vascular neoplasms, such as microvenular hemangioma, tufted angioma, and spindle cell hemangioma, which has made it helpful to distinguish ALHE from vascular malformations, as there is a negative staining pattern in the other entities...
January 2018: Dermatology Practical & Conceptual
https://www.readbyqxmd.com/read/29378601/tissue-expression-of-retinoic-acid-receptor-alpha-and-crabp2-in-metastatic-nephroblastomas
#14
Ana Paula Percicote, Gabriel Lazaretti Mardegan, Elizabeth Schneider Gugelmim, Sergio Ossamu Ioshii, Ana Paula Kuczynski, Seigo Nagashima, Lúcia de Noronha
BACKGROUND: Nephroblastoma or Wilms tumor is the most frequent kidney cancer in children and accounts for 98% of kidney tumors in this age group. Despite favorable prognosis, a subgroup of these patients progresses to recurrence and death. The retinoic acid (RA) pathway plays a role in the chemoprevention and treatment of tumors due to its effects on cell differentiation and its antiproliferative, anti-oxidant, and pro-apoptotic activities. Reports describe abnormal cellular retinoic acid-binding protein 2 (CRABP2) expression in neoplasms and its correlation with prognostic factors and clinical and pathological characteristics...
January 22, 2018: Diagnostic Pathology
https://www.readbyqxmd.com/read/29334535/simultaneous-presentation-of-wilms-tumor-and-contralateral-ganglioneuroma-in-a-child-case-report-and-literature-review
#15
Zuhal Bayramoglu, Ibrahim Adaletli, Emine Caliskan, Isin Kilicaslan, Cagla S Karaoglan, Alaattin Celik, Feryal Gun Soysal, Sema B Bay, Bulent Zulfikar
We demonstrate a 4-year-old girl who presented with progressive, asymmetrical, firm abdominal distention and was diagnosed with synchronous Wilms' tumor and left para-aortic ganglioneuroma (GN). Although synchronous tumors in the pediatric population are commonly associated with malignancy-predisposing syndromes, the patient in question was found to be otherwise healthy and had no clinical evidence nor family history of a syndrome. This case is the second one in the literature diagnosed with synchronous presentation of Wilms' tumor and GN in a previously healthy child...
January 12, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29333010/sinusoidal-obstruction-syndrome-during-treatment-for-wilms-tumor-a-life-threatening-complication
#16
Sidharth Totadri, Amita Trehan, Deepak Bansal, Richa Jain
Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Settings and Design: Retrospective file review conducted in a Pediatric Hematology-Oncology unit...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29320783/wt1-gene-mutation-p-r462w-in-a-46-xy-dsd-patient-from-egypt-with-gonadoblastoma-and-review-of-the-literature
#17
Inas Mazen, Heba Hassan, Alaa Kamel, Mona Mekkawy, Ken McElreavey, Mona Essawi
WT1 gene mutations have been described in 46,XY patients with ambiguous genitalia or complete gonadal dysgenesis with or without Wilms' tumor, nephropathy, gonadoblastoma, and other defects, e.g., cryptorchidism or hypospadias. p.R462W is a hot spot mutation in exon 9 and is the most common mutation in patients with Denys-Drash syndrome. However, in this study we report an Egyptian patient with a novel phenotype carrying the p.R462W mutation. We also review the heterogeneity of phenotypes of previously reported patients with the p...
2017: Sexual Development: Genetics, Molecular Biology, Evolution, Endocrinology, Embryology, and Pathology of Sex Determination and Differentiation
https://www.readbyqxmd.com/read/29301605/extrarenal-wilms-tumor-of-the-female-genital-system-a-case-report-and-literature-review
#18
Min-Min Cao, Cui-Ping Huang, Ya-Fen Wang, De-Mei Ma
Extrarenal Wilms' Tumors (ERWTs) are rare. There have been only 25 cases of ERWT arising from the female genital system reported in the literature. In this paper, we report a 60-year-old woman with a complaint of vaginal bleeding and a polypoid mass in the uterine cavity by sonography that was demonstrated as ERWT by pathology after resection. The pathological characteristics, histological origination, diagnosis, therapy and prognosis of ERWT in female reproductive system are discussed in this paper in the purpose of improving the diagnosis and therapy of this rare tumor...
December 30, 2017: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/29211618/outcome-and-prognostic-factors-in-stage-iii-favorable-histology-wilms-tumor-a-report-from-the-children-s-oncology-group-study-aren0532
#19
Conrad V Fernandez, Elizabeth A Mullen, Yueh-Yun Chi, Peter F Ehrlich, Elizabeth J Perlman, John A Kalapurakal, Geetika Khanna, Arnold C Paulino, Thomas E Hamilton, Kenneth W Gow, Zelig Tochner, Fredric A Hoffer, Janice S Withycombe, Robert C Shamberger, Yeonil Kim, James I Geller, James R Anderson, Paul E Grundy, Jeffrey S Dome
Background The National Wilms Tumor Study (NWTS) approach to treating stage III favorable-histology Wilms tumor (FHWT) is Regimen DD4A (vincristine, dactinomycin, and doxorubicin) and radiation therapy. Further risk stratification is required to improve outcomes and reduce late effects. We evaluated clinical and biologic variables for patients with stage III FHWT without combined loss of heterozygosity (LOH) at chromosomes 1p and 16q treated in the Children's Oncology Group protocol AREN0532. Methods From October 2006 to August 2013, 588 prospectively treated, centrally reviewed patients with stage III FHWT were treated with Regimen DD4A and radiation therapy...
January 20, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29187024/ossifying-renal-tumor-of-infancy-report-of-a-case-with-positive-wt1-immunohistochemistry-and-high-mitotic-index-and-review-of-the-literature
#20
Benoit Vaillancourt, Luc Oligny, Julie Déry, Julie Franc-Guimond, Dorothée Bouron-Dal Soglio
Ossifying renal tumor of infancy (ORTI) is a rare, benign pediatric tumor of the kidney. Since first reported by Chatten in 1980, 23 cases have been published. Previous authors have argued that ORTI might originate from nephrogenic rests, thereby sharing a pathogenic relationship with Wilms' tumor (WT). ORTI is characterized histologically by a population of polygonal osteoblast-like cells around an osteoid core and densely cellular component of blastemal-like or spindle cells. While the immunohistochemical profile of the cellular components has been reported, to the best of our knowledge, the status of WT1 expression has only been reported once, where it showed negative marking...
November 2017: Pediatric and Developmental Pathology
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