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Wilms Tumor-Nephroblastoma

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Rare Kidney Tumors

Abstract

Nephroblastoma, or Wilms tumor (WT), is the second most common extracranial solid tumor and the most common malignant renal tumor in children, accounting for 5% of all malignancies and 80% of all diagnosed renal cancers in children and teenagers. The overall survival has increased to over 90% due to international collaboration in cooperative group studies and employment of a multimodal treatment approach including surgery, radiation, and chemotherapy [1, 2]. The earliest of these studies, led by the National Wilms Tumor Study Group (NWTSG), which was superseded by the Children’s Oncology Group (COG) in 2002, and the International Society of Pediatric Oncology (SIOP), stratified patients based on tumor stage alone. However, over time, the discovery of additional clinical, histological, and biological prognostic factors has led to more precise treatments that augment therapy for patients at high risk of relapse while reducing therapy for patients at low risk of relapse.

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Authors and Affiliations

  1. Center for Cancer and Blood Disorders, Children’s National Health System, Washington, DC, USA

    Marie V. Nelson & Jeffrey S. Dome

  2. Centre de Cancérologie Pédiatrique, Hôpital d’Enfants de la Timone, Marseille, France

    Arnauld Verschuur

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  1. Marie V. Nelson
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Correspondence to Jeffrey S. Dome .

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Editors and Affiliations

  1. Department of Hematology and Oncology, Strasbourg University Hospital, Hopital Civil, Strasbourg, France

    Gabriel G. Malouf

  2. Department of Genitourinary Medical Oncology, MD Anderson Cancer Center, Houston, TX, USA

    Nizar M. Tannir

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Nelson, M.V., Verschuur, A., Dome, J.S. (2019). Wilms Tumor-Nephroblastoma. In: Malouf, G., Tannir, N. (eds) Rare Kidney Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-96989-3_2

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