Transcription factor Wilms' tumor 1 (WT1) was originally identified as a tumor suppressor for Wilms' tumor, but it is also overexpressed in a variety of cancer cells, suggesting a potential oncogenic function of WT1. It is important to understand molecular mechanisms underlying these dual functions of WT1 in tumorigenesis. In the current study, we report a synergistic role for signal transducer...

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...

CONTEXT Mutations of the p53 tumor suppressor gene are the most frequent alterations observed in human neoplasias affecting adults. In pediatric oncology, however, they have seldom been identified. Wilms' tumor is a renal neoplasia commonly occurring in children and is associated with mutations of the WT1 gene. The correlation between Wilms' tumor and alterations of the p53 gene has not been we...

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...

Several studies report autoantibody signatures in cancer. The majority of these studies analyzed adult tumors and compared the seroreactivity pattern of tumor patients with the pattern in healthy controls. Here, we compared the autoimmune response in patients with neuroblastoma and patients with Wilms tumor representing two different childhood tumors. We were able to differentiate untreated neu...

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...

Hematogones are immature normal B cell precursors with a characteristic immunophenotype profile on flow cytometry that typically do not express surface immunoglobulin light chains. In this report, we describe a case in which the hematogones exhibit light chain restriction. Our patient was a 4-year-old boy with a complicated medical history involving treatment for a presumed bilateral Wilms tumo...