Renal cell carcinoma is an uncommon tumor in pediatric population.It is important for the clinicians to know its diagnosis as the management of renal cell carcinoma RCC is different from Wilms Tumor which is the most common tumor encountered in children .The management is surgical resection with no proven role for chemo or radiotherapy so far. Here, we present a case of seven-year-old male chil...

Wilms' tumor is a type of kidney cancer that affects young children. Although a number of Wilms' tumor samples have been collected through international trials, the mechanisms underlying its progression remain challenging to determine. Extensive studies have identified somatic mutations at several loci in Wilms' tumorigenesis, including WT1, catenin, Wilms' tumor gene on the X chromosome (WTX) ...

Studies on the loss of heterozygosity (LOH) in human malignancies have shown that a number of different chromosomal regions associated with putative tumor suppressor genes may be involved in any one given tumor. We have carried out a similar study on Wilms' tumor using a range of DNA markers for a number of tumor suppressor regions. We tested a total of 44 Wilms' tumors including material from ...

Wilms' tumor, or nephroblastoma, is the most common pediatric renal cancer. The tumors morphologically resemble embryonic kidneys with a disrupted architecture and are associated with undifferentiated metanephric precursors. Here, we discuss genetic and epigenetic findings in Wilms' tumor in the context of renal development. Many of the genes implicated in Wilms' tumorigenesis are involved in t...

Background: Wilms’ tumor is a renal malignancy commonly occurring in childhood, with classic histologic features. Extrarenal Wilms’ tumor is extremely rare, and only a few isolated cases have been described in the literature in the retroperitoneum, inguinal region, uterus, cervix, ovaries, skin, mediastinum, and chest wall. Case: A 14-month-old child presented with abdominal distention and a pa...

We have analyzed the chromosome 6q21 breakpoint of a non-constitutional t(6;15)(q21;q21) rearrangement in sporadic Wilms' tumor. This identified a novel gene encoding a protein with six N-terminal ankyrin repeats linked to a C-terminal HECT ubiquitin-protein ligase domain. We therefore designated this gene HACE1 (HECT domain and Ankyrin repeat Containing E3 ubiquitin-protein ligase 1). HACE1 is...

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...

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...

Article history: Received 28 June 2016 Accepted 28 June 2016 Available online 4 July 2016 pathway) and genetic progression (e.g., the Fearon-Vogelstein pathway Fearon and Vogelstein, 1990). Steps in adult tumor pathogenesis was suggested by Armitage and Doll 60 years ago (Armitage and Doll, 1954), with around 7 steps. The question then for pediatric tumors, is how do steps occur at such a young...