Wilms’ Tumor in Children: An Overview

Abstract

Wilms’ tumor is the most frequently occurring renal tumor in children and is one of the most treatment-responsive tumors. A tumor-suppressor gene and other genetic abnormalities have been implicated in its etiology. In addition, patients with several congenital anomalies, such as Beckwith-Wiedemann syndrome, WAGR syndrome, and Denys-Drash syndrome, have an increased risk of Wilms’ tumor. Previously, a three-drug chemotherapy regimen with surgery and radiotherapy was used with patients in all stages. Now, patients with early-stage Wilms’ tumor are treated with a two-drug regimen without radiotherapy, whereas those in advanced stages still receive the three-drug regimen and radiotherapy. Two large collaborative groups – the National Wilms’ Tumor Study Group (NWTS) in the United States and the International Society of Pediatric Oncology (SIOP) in Europe – are involved in Wilms’ tumor management, which differs in some aspects. Multimodality treatment has been used successfully, and in Europe preoperative strategies are used as well. As the survival rate has now reached 90%, the primary objectives of the physician are to perform nephron-sparing surgery in selected cases and to reduce the dosage and duration of chemotherapy and radiotherapy in appropriate cases. Other renal tumors occur rarely, but have also been treated successfully in the last decade.

Sphingosine 1-Phosphate in Renal Diseases

“…Wilms tumor is the most prevalent renal malignant tumor in children. But fortunately, it is also one of the most treatment-responsive tumors (reviewed in [88]). Thus, several therapeutic options are available to treat Wilms tumor successfully.…”

Surgical principles in pediatric oncology

“…Kaval trombüs cerrahi öncesinde bilinmezse nefrektomi sırasında emboli ve mortaliteye neden olabilir (2) bun farklı tedavi yaklaşımı bulunmaktadır. Bu gruplar, Kuzey Amerika ülkelerini kapsayan Ulusal Wilms Tümörü Çalışma Grubu (National Wilms' Tumor Study Group (NWTSG) günümüzde Children's Oncology Group (COG)'un bir parçasıdır) ile Avrupa'da Société Internationale d'Oncologie Pédiatrique (SIOP)'dir (2,3) . Ülkemizde ise olgular Türk Pediatrik Onkoloji Grubu (TPOG) protokolüne göre sağaltıl-maktadır (4) .…”

Morphological Features of Wilms’ Tumour in a Tertiary Health Care Institution: Our Findings

“…This assertion can be corroborated by studies done in the sub-Saharan Africa by Ekense et al [2,3] which showed that Wilms' tumour is the most common childhood malignancy in Sub-Saharan Africa. Despite being a malignant tumour, a survival rate of over 90% is now seen today (compared to 30% in the thirties) and this is an evidence of the success of collaborative trials and the use of multimodal therapy [4][5][6]. Generally, childhood renal tumours are largely of embryonic origin, with rapidity of growth and a better response to therapy [1].…”

“…Generally, childhood renal tumours are largely of embryonic origin, with rapidity of growth and a better response to therapy [1]. Black children have a 2.5 times increased incidence over their white counterparts and the sex ratio is close to 1 [4]. Wilms' tumour is also associated with a number of recognised syndromes including WAGR, Beckwith-Wiedemann and Denys-Drash syndromes [1].…”