Role of dactinomycin in the improved survival of children with Wilms' tumor

JAMA ◽  
1966 ◽  
Vol 195 (12) ◽  
pp. 1005-1009 ◽  
Author(s):  
D. J. Fernbach
Keyword(s):  
Wilms Tumor

WILMS' TUMOR AND CONGENITAL HEMIHYPERTROPHY: REPORT OF FIVE NEW CASES AND REVIEW OF LITERATURE

PEDIATRICS ◽  
1967 ◽  
Vol 40 (5) ◽  
pp. 886-899
Author(s):  
Joseph F. Fraumeni ◽  
Clementina F. Geiser ◽  
Miriam D. Manning
Keyword(s):  
Cancer Research ◽  
Adrenal Cortex ◽  
Wilms Tumor ◽  
Cytogenetic Study ◽  
Congenital Defect ◽  
Familial Occurrence ◽  
Review Of Literature ◽  
Childhood Neoplasms ◽  
Congenital Hemihypertrophy

Among 225 patients with Wilms' tumor seen at the Children's Cancer Research Foundation in Boston, 7 had congenital hemihypertrophy (a frequency of 1:32), bringing to 26 the number of cases reported in the literature with this association. In one child the cytogenetic study of leukocyte cultures revealed elongation of the long arms of both No. 16 chromosomes; each parent and two of four siblings had a similar anomaly affecting one chromosome of pair 16. Dermatoglyphics on this patient and three others in the series were unremarkable, as were studies of urinary gonadotropin excretion. From a review of all cases reported with Wilms' tumor and hemihypertrophy, little was found to indicate a relationship to other disorders, such as Silver's syndrome or neurofibromatosis, in which hemihypertrophy has been described. A role of inheritance was suggested in our series by one patient who had a sibling with hemihypertrophy, the seventh reported familial occurrence of this congenital defect. From the sparse evidence available, it would appear that the origins of hemihypertrophy are heterogeneous and include genic, chromosomal, and other factors which are presently obscure. The association between hemihypertrophy and Wilms' tumor may reflect common etiologic factors or a pre-neoplastic anlage in "hemihypertrophic" kidneys. Since hemihypertrophy seems to be related also to childhood neoplasms originating in the adrenal cortex and liver, further research on this anomaly should enhance our understanding of oncogenic mechanisms.

THE ROLE OF PREOPERATIVE CHEMOTHERAPY IN THE MANAGEMENT OF WILMS' TUMOR

2000 ◽  
Vol 27 (3) ◽  
pp. 443-454 ◽  
Author(s):  
Norbert Graf ◽  
Marie-France Tournade ◽  
Jan de Kraker
Keyword(s):  
Preoperative Chemotherapy ◽  
Wilms Tumor

Multimodal management of recurrent Wilms' tumor: The role of radiation therapy

1996 ◽  
Vol 27 (3) ◽  
pp. 179-184
Author(s):  
William W. Thoms ◽  
Roger Vega ◽  
Carlos Abramowsky ◽  
Richard Ricketts ◽  
Brad Wyly
Keyword(s):  
Radiation Therapy ◽  
Wilms Tumor ◽  
Multimodal Management

scholarly journals TP53 rs1042522 C>G polymorphism and Wilms tumor susceptibility in Chinese children: a four-center case–control study

2019 ◽  
Vol 39 (1) ◽  
Author(s):  
Peng Liu ◽  
Zhenjian Zhuo ◽  
Wenya Li ◽  
Jiwen Cheng ◽  
Haixia Zhou ◽  
...  
Keyword(s):  
Significant Relationship ◽  
Wilms Tumor ◽  
Tp53 Gene ◽  
Chinese Children ◽  
Logistic Regression Models ◽  
Tumor Susceptibility ◽  
Tumor Risk ◽  
Larger Sample ◽  
Control Study

Abstract Wilms tumor is the most common renal malignancy that occurs in children. TP53 gene is considered as a tumor-suppressing gene through controlling cell growth. TP53 gene rs1042522 C>G (Arg72Pro) polymorphism is widely investigated in various types of cancers. However, it is not established if TP53 rs1042522 C>G polymorphism is a candidate variant for Wilms tumor risk. The aim of the study was to determine whether TP53 rs1042522 C>G polymorphism is responsible for the risk of Wilms tumor in Chinese children. All subjects (355 cases and 1070 controls) from four centers of China were genotyped for rs1042522 C>G polymorphism. The effect of rs1042522 C>G polymorphism on Wilms tumor prevalence was analyzed using logistic regression models. We failed to detect a significant relationship between rs1042522 C>G polymorphism and Wilms tumor risk. Further stratification analysis also could not detect a significant relationship. We conclude that TP53 rs1042522 C>G polymorphism might not have enough impact on the risk of Wilms tumor. More validation study with larger sample size will be required to better define the role of TP53 rs1042522 C>G polymorphism in Wilms tumor risk.

scholarly journals The Wilms’ Tumor Suppressor Gene (wt1) Product Regulates Dax-1 Gene Expression during Gonadal Differentiation

1999 ◽  
Vol 19 (3) ◽  
pp. 2289-2299 ◽  
Author(s):  
Jungho Kim ◽  
Dirk Prawitt ◽  
Nabeel Bardeesy ◽  
Elena Torban ◽  
Caroline Vicaner ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Receptor Gene ◽  
Suppressor Gene ◽  
Gonadal Development ◽  
Early Event ◽  
Testicular Development ◽  
Gonadal Differentiation ◽  
Mobility Shift ◽  
Drash Syndrome

ABSTRACT Gonadal differentiation is dependent upon a molecular cascade responsible for ovarian or testicular development from the bipotential gonadal ridge. Genetic analysis has implicated a number of gene products essential for this process, which include Sry, WT1, SF-1, and DAX-1. We have sought to better define the role of WT1 in this process by identifying downstream targets of WT1 during normal gonadal development. We have noticed that in the developing murine gonadal ridge, wt1 expression precedes expression of Dax-1, a nuclear receptor gene. We document here that the spatial distribution profiles of both proteins in the developing gonad overlap. We also demonstrate that WT1 can activate the Dax-1 promoter. Footprinting analysis, transient transfections, promoter mutagenesis, and mobility shift assays suggest that WT1 regulates Dax-1via GC-rich binding sites found upstream of the Dax-1 TATA box. We show that two WT1-interacting proteins, the product of a Denys-Drash syndrome allele of wt1 and prostate apoptosis response-4 protein, inhibit WT1-mediated transactivation ofDax-1. In addition, we demonstrate that WT1 can activate the endogenous Dax-1 promoter. Our results indicate that the WT1–DAX-1 pathway is an early event in the process of mammalian sex determination.

The Role of Computerized Tomography in the Diagnosis and Management of Patients With Bilateral Wilms Tumor

1983 ◽  
Vol 130 (6) ◽  
pp. 1160-1162 ◽  
Author(s):  
Mervyn D. Cohen ◽  
Thomas Weber ◽  
John A. Smith ◽  
Jeffrey I. Reider
Keyword(s):  
Computerized Tomography ◽  
Wilms Tumor ◽  
Diagnosis And Management ◽  
Bilateral Wilms Tumor

scholarly journals Androgen Promotes Differentiation of PLZF+ Spermatogonia pool via Indirect Regulatory Pattern

2018 ◽  
Author(s):  
Jingjing Wang ◽  
Jinmei Li ◽  
Yunzhao Gu ◽  
Qin Xia ◽  
Weixiang Song ◽  
...  
Keyword(s):  
Germ Cells ◽  
Sertoli Cells ◽  
Culture System ◽  
Molecular Mechanisms ◽  
Wilms Tumor ◽  
Post Partum ◽  
Mice Model ◽  
Androgen Action ◽  
Wilms Tumor 1

AbstractAndrogen signaling plays a pivotal role in spermatogenesis, but the molecular mechanisms underlying androgen action in this process are unclear. Specifically, it is unknown if the androgen receptor (AR) is expressed in germ cells. Thus it’s interesting to reveal how androgen induces differentiation of spermatogonial progenitor cells (SPCs) in the niche. Here we observed the AR is primarily expressed in pre-spermatogonia of mice 2 days post partum (dpp), absent before spermatogenesis onset, and then expressed in surrounding Sertoli cells. Then we examined a regulatory role of the AR in spermatogenesis using a SPCs-Sertoli cells co-culture system, and demonstrated that androgen negatively regulated Plzf (the gene for stemness maintenance of SPCs). Additionally, we identified Gata2 as a target of AR in Sertoli cells, and demonstrated that Wilms tumor 1 (WT1) and β1-integrin as two putative intermediate molecules to transfer differentiation signals to SPCs, which was further verified using androgen pharmacological-deprivation mice model. These results demonstrate a regulatory pattern of androgen in SPCs niche in an indirect way via multiple steps of signal transduction.

scholarly journals circ0093740 Promotes Tumor Growth and Metastasis by Sponging miR-136/145 and Upregulating DNMT3A in Wilms Tumor

2021 ◽  
Vol 11 ◽  
Author(s):  
Juan Cao ◽  
Zhongying Huang ◽  
Shunling Ou ◽  
Feiqiu Wen ◽  
Guocheng Yang ◽  
...  
Keyword(s):  
Tumor Growth ◽  
Molecular Mechanism ◽  
Wilms Tumor ◽  
Circular Rnas ◽  
Migration Ability ◽  
Rna Immunoprecipitation ◽  
New Treatment ◽  
Tumor Growth And Metastasis ◽  
And Migration

As a research hotspot, circular RNAs (circRNAs) is one type of non-coding RNAs which have many different functions in biological processes. However, there is lack of study investigating the underlying molecular mechanism and the potential roles of circRNAs in Wilms tumor. We conducted a high-throughput microarray sequencing to screen differentially expressed circRNAs in Wilms tumor. A novel circRNA (circ0093740) was identified as a frequently upregulated circRNA in Wilms tumor cells and tissues. Suppression of circ0093740 remarkably inhibited the proliferation and migration ability in Wilms tumor, validated by several experiments. The molecular mechanism of circ0093740 was investigated by luciferase assays and RNA immunoprecipitation assays. The results revealed that circ0093740 promotes the growth and migration ability by sponging miR-136/145 and upregulating DNMT3A. In conclusion, our study discovered the biological role of the circ0093740-miR-136/145-DNMT3A axis in Wilms tumor growth and metastasis which is important for developing new treatment strategy.

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