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Biological Drivers of Wilms Tumor Prognosis and Treatment

Vanderbilt University School of Medicine, Vanderbilt University, Nashville, TN 37232, USA
Department of Pediatrics, Division of Pediatric Hematology-Oncology, Vanderbilt University Medical Center Nashville, TN 37232, USA
Department of Pediatric Surgery, Vanderbilt University Medical Center Nashville, TN 37232, USA
Author to whom correspondence should be addressed.
Children 2018, 5(11), 145;
Received: 29 September 2018 / Revised: 16 October 2018 / Accepted: 18 October 2018 / Published: 26 October 2018
(This article belongs to the Special Issue Pediatric Surgical Oncology)
PDF [227 KB, uploaded 26 October 2018]


Prior to the 1950s, survival from Wilms tumor (WT) was less than 10%. Today, a child diagnosed with WT has a greater than 90% chance of survival. These gains in survival rates from WT are attributed largely to improvements in multimodal therapy: Enhanced surgical techniques leading to decreased operative mortality, optimization of more effective chemotherapy regimens (specifically, dactinomycin and vincristine), and inclusion of radiation therapy in treatment protocols. More recent improvements in survival, however, can be attributed to a growing understanding of the molecular landscape of Wilms tumor. Particularly, identification of biologic markers portending poor prognosis has facilitated risk stratification to tailor therapy that achieves the best possible outcome with the least possible toxicity. The aim of this review is to (1) outline the specific biologic markers that have been associated with prognosis in WT and (2) provide an overview of the current use of biologic and other factors to stratify risk and assign treatment accordingly. View Full-Text
Keywords: Wilms tumor; nephroblastoma; tumor biology; biomarkers; therapy Wilms tumor; nephroblastoma; tumor biology; biomarkers; therapy
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

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Phelps, H.M.; Kaviany, S.; Borinstein, S.C.; Lovvorn, H.N., III. Biological Drivers of Wilms Tumor Prognosis and Treatment. Children 2018, 5, 145.

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