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. 2023 Sep;70 Suppl 6(Suppl 6):e30586.
doi: 10.1002/pbc.30586. Epub 2023 Jul 21.

Children's Oncology Group's 2023 blueprint for research: Renal tumors

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Children's Oncology Group's 2023 blueprint for research: Renal tumors

James I Geller et al. Pediatr Blood Cancer. 2023 Sep.

Abstract

Every year, approximately 600 infants, children, and adolescents are diagnosed with renal cancer in the United States. In addition to Wilms tumor (WT), which accounts for about 80% of all pediatric renal cancers, clear cell sarcoma of the kidney, renal cell carcinoma, malignant rhabdoid tumor, as well as more rare cancers (other sarcomas, rare carcinomas, lymphoma) and benign tumors can originate within the kidney. WT itself can be divided into favorable histology (FHWT), with a 5-year overall survival (OS) exceeding 90%, and anaplastic histology, with 4-year OS of 73.7%. Outcomes of the other pediatric renal cancers include clear cell sarcoma (5-year OS: 90%), malignant rhabdoid tumor (5-year OS: 10% for stages 3 and 4), and renal cell carcinoma (4-year OS: 84.8%). Recent clinical trials have identified novel biological prognostic markers for FHWT, and a series of Children's Oncology Group (COG) trials have demonstrated improving outcomes with therapy modification, and opportunities for further care refinement.

Keywords: Wilms tumor; clear cell sarcoma; renal; rhabdoid tumor; tumors.

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Figures

Figure 1:
Figure 1:
Evoluation of risk stratification factors for Wilms tumor.

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References

    1. Fernandez CV, Mullen EA, Chi YY, et al.: Outcome and Prognostic Factors in Stage III Favorable-Histology Wilms Tumor: A Report From the Children's Oncology Group Study AREN0532. J Clin Oncol 36:254–261, 2018 - PMC - PubMed
    1. Fernandez C et al.: Clinical Outcome and Biological Predictors of Relapse After Nephrectomy Only for Very Low-Risk Wilms Tumor; Annals of Surgery, Volume 265, Number 4, April 2017. Dix AREN0533 - PMC - PubMed
    1. Dix D et al.,: Augmentation of Therapy for Combined Loss of Heterozygosity 1p and 16q in Favorable Histology Wilms Tumor: A Children’s Oncology Group AREN0532 and AREN 0533 Study Report; JCO, Aug 2019. - PMC - PubMed
    1. Dix D et al.: Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report from the Children’s Oncology Group AREN0533 Study: JCO, April 2018. - PMC - PubMed
    1. Daw NC, Chi YY, Kalapurakal JA, Kim Y, Hoffer FA, Geller JI, Perlman EJ, Ehrlich PF, Mullen EA, Warwick AB, Grundy PE, Paulino AC, Gratias E, Ward D, Anderson JR, Khanna G, Tornwall B, Fernandez CV, Dome JS; AREN0321 Study Committee. Activity of Vincristine and Irinotecan in Diffuse Anaplastic Wilms Tumor and Therapy Outcomes of Stage II to IV Disease: Results of the Children's Oncology Group AREN0321 Study. J Clin Oncol. 2020. May 10;38(14):1558–1568. - PMC - PubMed

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