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      Current treatment for Wilms tumor: COG and SIOP standards

      , , , , ,
      World Journal of Pediatric Surgery
      BMJ

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          Abstract

          Background

          Wilms tumor (WT) is the most common renal malignant tumor in children. It occurs primarily at preschool age. The purpose of this review is to present current standards of diagnosis and treatment of WT around the world.

          Data sources

          All the recent literature on diagnosis and treatment of WT were searched and reviewed.

          Results

          Most cases with WT are sporadic. The current survival in patients with WT is high (90%). Involvement of mutidisciplinary collaborative groups in the diagnosis and treatment of WT. National Wilms Tumor Study Group (NWTSG)/Children’s Oncology Group (COG) and The International Society of Paediatric Oncology (SIOP) are two major guidelines used for the current management of WT worldwide. The major difference exists in the two guidelines is the timing of surgery: SIOP recommends using preoperative chemotherapy, and NWTSG/COG prefers using primary surgery before any adjuvant treatments.

          Conclusions

          Most patients with WT have good overall survival outcomes. Further studies should be highlighted on how to use chemotherapy and radiotherapy under more accurate risk-stratified strategies. Surgeons must be more focusing on how to maximize preoperative and postoperative treatment possibilities for achieving optimal results of patients with WT.

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          Most cited references20

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          The UMBRELLA SIOP–RTSG 2016 Wilms tumour pathology and molecular biology protocol

          On the basis of the results of previous national and international trials and studies, the Renal Tumour Study Group of the International Society of Paediatric Oncology (SIOP–RTSG) has developed a new study protocol for paediatric renal tumours: the UMBRELLA SIOP–RTSG 2016 protocol (the UMBRELLA protocol). Currently, the overall outcomes of patients with Wilms tumour are excellent, but subgroups with poor prognosis and increased relapse rates still exist. The identification of these subgroups is of utmost importance to improve treatment stratification, which might lead to reduction of the direct and late effects of chemotherapy. The UMBRELLA protocol aims to validate new prognostic factors, such as blastemal tumour volume and molecular markers, to further improve outcome. To achieve this aim, large, international, high-quality databases are needed, which dictate optimization and international harmonization of specimen handling and comprehensive sampling of biological material, refine definitions and improve logistics for expert review. To promote broad implementation of the UMBRELLA protocol, the updated SIOP–RTSG pathology and molecular biology protocol for Wilms tumours has been outlined, which is a consensus from the SIOP–RTSG pathology panel.
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            Epidemiology of Wilms tumor

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              Children's Oncology Group's 2013 blueprint for research: renal tumors.

              Renal malignancies are among the most prevalent pediatric cancers. The most common is favorable histology Wilms tumor (FHWT), which has 5-year overall survival exceeding 90%. Other pediatric renal malignancies, including anaplastic Wilms tumor, clear cell sarcoma, malignant rhabdoid tumor, and renal cell carcinoma, have less favorable outcomes. Recent clinical trials have identified gain of chromosome 1q as a prognostic marker for FHWT. Upcoming studies will evaluate therapy adjustments based on this and other novel biomarkers. For high-risk renal tumors, new treatment regimens will incorporate biological therapies. A research blueprint, viewed from the perspective of the Children's Oncology Group, is presented. Copyright © 2012 Wiley Periodicals, Inc.
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                Author and article information

                Contributors
                Journal
                World Journal of Pediatric Surgery
                World Jnl Ped Surgery
                BMJ
                2516-5410
                February 21 2020
                February 2020
                September 27 2019
                September 2019
                : 2
                : 3
                : e000038
                Article
                10.1136/wjps-2019-000038
                52c43653-4de3-4ffb-a935-5bcad113745f
                © 2019
                History

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