Like testicular cancer in adulthood, the Wilms' tumor or nephroblastoma in childhood is an example of a curable malignant disease. The review by Huszno et al. in this Journal gives an overview concerning diagnosis and treatment of adult Wilms' tumors [1]. This tumor entity was first described in 1814 by Rance and in 1899 Max Wilms, a German surgeon, described this tumor in detail [2]. Beckwith ...

From 2003 to 2013, 55 patients (median age 3.3 years; 29 males, 26 females) with unresectable, metastatic, or diffuse anaplastic histology (AH) Wilms tumor were treated with neoadjuvant transcatheter arterial chemoembolization (TACE) and systemic chemotherapy. Characteristics In: Wilms Tumor. Marry M. van den Heuvel-Eibrink (Editor) ISBN: 978-0-9944381-1-9; Doi: http://dx.doi.org/10.15586/codon...

PURPOSE A number of studies have indicated that the tumor proliferation marker MIB-1 and cell cycle inhibitor p27(Kip1) expression are of prognostic importance in a variety of cancers. The present study was performed to evaluate the prognostic value of these molecules in Wilms' tumors. EXPERIMENTAL DESIGN MIB-1 and p27(Kip1) expressions were investigated by the means of immunohistochemical an...

Metanephric adenofibroma is a rare, biphasic, benign tumor containing both stromal and epithelial components and could be potentially mistaken as Wilms' tumor (WT). We present a 5-year-old girl who was suspected to have metastatic Wilms' tumor on radiological investigations/tru-cut biopsy and had received neoadjuvant chemotherapy, but postoperatively final histopathology revealed it as metaneph...

Insulin-like growth factor-II (IGF-II) is thought to play a critical role in the development of embryonic tumors such as Wilms' tumor. However, despite highly elevated IGF-II mRNA levels in tumors, IGF-II is not elevated in the serum of patients with Wilms' tumors. Recently high molecular weight forms of IGF-II ('big'- or pro-IGF-II) have been found to be produced by some tumors. In order to pr...

Human insulin-like growth factor II gene (IGF2) is overexpressed, and its imprinting is disrupted in many tumors, including Wilms' tumor. A transcript that is antisense to IGF2, IGF2-antisense (IGF2-AS), is transcribed from within IGF2 in a reverse orientation. This transcript is also maternally imprinted and overexpressed in Wilms' tumor. IGF2-AS was detected as a 2.2 kb mRNA in Hep 3B cells b...

Although tumour suppressor gene hypermethylation is a universal feature of cancer cells, little is known about the necessary molecular triggers. Here, we show that Wilms' tumour 1 (WT1), a developmental master regulator that can also act as a tumour suppressor or oncoprotein, transcriptionally regulates the de novo DNA methyltransferase 3A (DNMT3A) and that cellular WT1 levels can influence DNA...

BACKGROUND Although the NWTSG and SIOP studies have included the largest number of patients, several individual institutions have made likewise important contributions to the optimization of Wilms' tumor therapy. The purpose of this study is to present our personal experience obtained in the last 42 years by treating Wilms' tumor in childhood. PATIENTS AND METHODS Throughout the period 1965-2...

Wilms' tumor or nephroblastoma is believed to arise from embryonic nephrogenic rests of multipotent cells that fail to terminally differentiate into epithelium and continue to proliferate. The WT1 tumor suppressor gene, a transcription factor controlling the mesenchymal-epithelial transition in renal development, is mutated in 10% to 15% of Wilms' tumors. This potentially explains the disordere...

BACKGROUND Children with multicystic kidney disease (MCKD) are increasingly managed conservatively and are followed up throughout childhood because they are perceived to be at increased risk of developing Wilms' tumour. With this risk still poorly defined and somewhat controversial, the strategy and the duration of follow up do not seem to be based on evidence. METHODS Systematic review of th...