More than 25 years after introducing preoperative chemotherapy for Wilms' tumor, the benefits of this approach are well known. The preoperative protocol results in easier operations with significantly fewer tumor ruptures during surgery and a favorable stage distribution. Acute toxicity and late effects are minimized without jeopardizing disease-free and overall survival. Future clinical trials of Wilms' tumor should seek additional risk factors to stratify and individualize treatment. These prognostic factors will improve the cure rates for high-risk patients by intensifying therapy and the quality of life for children with more favorable prognosis by lowering therapy to a minimum. As is true for radical surgery, partial nephrectomy in unilateral disease must be evaluated in carefully selected patients according to clear and well-defined indications. Molecular genetic studies should increase understanding of Wilms' tumor, influencing treatment and outcome.

Download full-text PDF

Source
http://dx.doi.org/10.1016/s0094-0143(05)70092-6DOI Listing

Publication Analysis

Top Keywords

wilms' tumor
16
preoperative chemotherapy
8
tumor
5
role preoperative
4
chemotherapy management
4
wilms'
4
management wilms'
4
tumor siop
4
siop studies
4
studies international
4

Similar Publications

Paediatric renal tumours: an update on challenges and recent developments.

Virchows Arch

January 2025

Histology Laboratory, Children's Health Ireland, Dublin, Ireland.

Paediatric renal tumours include a broad range of neoplasms which largely differ, but also overlap to a smaller extent, with adult kidney cancer. These include the embryonal tumour nephroblastoma, which accounts for the majority of cases of kidney cancer in the first decade of life and, despite boasting a cure in ~ 90% cases, still presents clinical challenges in a small proportion of cases. A variety of less common mesenchymal tumours, including the mostly indolent congenital mesoblastic nephroma, clear cell sarcoma of kidney which continues to be associated with poor outcomes for higher stage disease, and the typically lethal malignant rhabdoid tumour, form the bulk of the remaining presentations in the first decade.

View Article and Find Full Text PDF

Background: Several studies have investigated long-term kidney outcomes in survivors of Wilms tumor (WT). However, many have small sample sizes, and there is a wide variation in reported outcomes. The aim of this study is to investigate the long-term kidney outcomes in survivors of WT (S-WT), including those patients considered to be at high risk for poor kidney outcomes, and using updated estimated glomerular filtration rate (eGFR) equations.

View Article and Find Full Text PDF

Establishing genotype-phenotype correlations in disorders of hereditary endocrine neoplasia is important for clinical screening, genetic counseling, prognostication, surveillance, and surgical strategy, and may also provide clues about disease pathogenesis. Important genotype-phenotype correlations are recognized, for example, in pheochromocytoma/paraganglioma and multiple endocrine neoplasia type 2A. The presence of such correlations has been less clear in other familial endocrine disorders associated with primary hyperparathyroidism including multiple endocrine neoplasia type 1 (MEN1), and the hyperparathyroidism-jaw tumor syndrome (HPT-JT).

View Article and Find Full Text PDF

Background: Circulating tumour cells (CTCs) and tumour-derived extracellular vesicles (tdEVs) have great potential for monitoring therapy response and early detection of tumour relapse, facilitating personalized adjuvant therapeutic strategies. However, their low abundance in peripheral blood limits their informative value. In this study, we explored the presence of CTCs and tdEVs collected intraoperatively from a tumour-draining vein (DV) and via a central venous catheter (CVC) prior to tumour resection.

View Article and Find Full Text PDF

The Wilms' tumor suppressor WT1 is essential for the development of the heart, among other organs such as the kidneys and gonads. The Wt1 gene encodes a zinc finger transcription factor that regulates proliferation, cellular differentiation processes, and apoptosis. WT1 is also involved in cardiac homeostasis and repair.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!