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...

Wilms tumor is found in 1 in 10,000 children and most commonly presents in asymptomatic toddlers whose care givers notice a nontender abdominal mass in the right upper quadrant. This case of Wilms tumor presented as a critically ill eleven-year old with significant tachypnea, dyspnea, vague abdominal pain, intermittent emesis, new onset seizure, metabolic acidosis, and hypoxemia. This is the fi...

Renal tumors other than Wilms’ tumor are infrequent in childhood. Wilms’ tumors account for 6% to 7% of childhood cancer, whereas the remaining renal tumors account for less than l%.27 The most common nonWilms‘ tumors are clear cell sarcoma of the kidney, rhabdoid tumor of the kidney (both formerly considered unfavorable Wilms’ tumor variants but now considered separate tumors), renal cell carc...

BACKGROUND Loss of imprinting (LOI) of the H19/IGF2 domain is a common feature of Wilms tumour. The GTL2/DLK1 domain is also imprinted and is structurally similar to H19/IGF2. The question arises as to whether DLK1 also undergoes LOI in Wilms tumour, or whether the LOI mechanism is restricted to the H19/IGF2 domain. AIM To investigate the imprinting status of DLK1 in Wilms tumours with IGF2 L...

W ilms tumour (WT) is the most common malignant neoplasm of the kidney in childhood and accounts for approximately 8% of all childhood solid tumours. 2 Fetal rhabdomyomatous nephroblastoma (FRN) is a histological variant of WT characterised by a predominance of rhabdomyogenic components. Clinically, WT of the FRN type presents as a huge mass in younger patients and about 30% of them have bilate...