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Review
. 2021 Aug 1;19(8):978-985.
doi: 10.6004/jnccn.2021.7070.

Impact of the First Generation of Children's Oncology Group Clinical Trials on Clinical Practice for Wilms Tumor

Jeffrey S Dome  1 , Elizabeth A Mullen  2 , David B Dix  3 , Eric J Gratias  4 , Peter F Ehrlich  5 , Najat C Daw  6 , James I Geller  7 , Murali Chintagumpala  8 , Geetika Khanna  9 , John A Kalapurakal  10 , Lindsay A Renfro  11 , Elizabeth J Perlman  12 , Paul E Grundy  13 , Conrad V Fernandez  14
Affiliations
Review

Impact of the First Generation of Children's Oncology Group Clinical Trials on Clinical Practice for Wilms Tumor

Jeffrey S Dome et al. J Natl Compr Canc Netw. .

Abstract

Refinements in surgery, radiation therapy, and chemotherapy since the mid-20th century have resulted in a survival rate exceeding 90% for patients with Wilms tumor (WT). Although this figure is remarkable, a significant proportion of patients continue to have event-free survival (EFS) estimates of <75%, and nearly 25% of survivors experience severe chronic medical conditions. The first-generation Children's Oncology Group (COG) renal tumor trials (AREN '0'), which opened to enrollment in 2006, focused on augmenting treatment regimens for WT subgroups with predicted EFS <75% to 80%, including those with the adverse prognostic marker of combined loss of heterozygosity (LOH) at chromosomes 1p/16q, pulmonary metastasis with incomplete lung nodule response after 6 weeks of chemotherapy, bilateral disease, and anaplastic histology. Conversely, therapy was reduced for patient subgroups with good outcomes and potential for long-term toxicity, such as those with lung metastasis with complete lung nodule response after 6 weeks of chemotherapy. This article summarizes the key findings of the first-generation COG renal tumor studies and their implications for clinical practice.

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Figures

Figure 1.
Figure 1.. Classification of WT subsets according to potential for late effects and event-free survival at the beginning of the AREN ‘0’ generation of studies.
Abbreviations: AHWT, anaplastic histology Wilms tumor; CR, complete response; FHWT, favorable histology Wilms tumor; IR, incomplete response; LOH, combined loss of heterozygosity at chromosomes 1p and 16q; WT, Wilms tumor. aExcept for patients with very low risk WT, as described in the text, who were candidates to receive no adjuvant therapy.
Figure 2.
Figure 2.. Organization of the first-generation COG AREN ‘0’ Renal Tumor Studies. The AREN03B2 Classification and Banking study served as a required gateway of entry to 1 of 4 frontline therapeutic studies.
Abbreviations: AHWT, anaplastic histology Wilms tumor; CCSK, clear cell sarcoma of the kidney; FHWT, favorable histology Wilms tumor; LOH, combined loss of heterozygosity at chromosomes 1p and 16q; MRT, malignant rhabdoid tumor; RCC, renal cell carcinoma; WT, Wilms tumor.
Figure 3.
Figure 3.. Risk classification and banking flow diagram for AREN03B2.
Abbreviations: FHWT, favorable histology Wilms tumor; LOH, loss of heterozygosity testing for chromosomes 1p and 16q.
See this image and copyright information in PMC

Comment in

References

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