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. 2020 Jun 15;126(12):2866-2871.
doi: 10.1002/cncr.32855. Epub 2020 Apr 8.

Outcome analysis of stage I epithelial-predominant favorable-histology Wilms tumors: A report from Children's Oncology Group study AREN03B2

Lauren N Parsons  1 , Elizabeth A Mullen  2 , James I Geller  3 , Yueh-Yun Chi  4 , Geetika Khanna  5 , Richard D Glick  6 , Jennifer H Aldrink  7 , Kelly L Vallance  8 , Yeonil Kim  4 , Conrad V Fernandez  9   10 , Jeffrey S Dome  11 , Elizabeth J Perlman  12
Affiliations

Outcome analysis of stage I epithelial-predominant favorable-histology Wilms tumors: A report from Children's Oncology Group study AREN03B2

Lauren N Parsons et al. Cancer. .

Abstract

Background: Stage I epithelial-predominant favorable-histology Wilms tumors (EFHWTs) have long been suspected to have an excellent outcome. This study investigates the clinical and pathologic features of patients with stage I EFHWTs to better evaluate the potential for a reduction of chemotherapy and its associated toxicity.

Methods: All patients registered in the Children's Oncology Group (COG) AREN03B2 study between 2006 and 2017 with stage I EFHWTs were identified. EFHWTs were defined as tumors with at least 66% epithelial differentiation, regardless of the degree of differentiation. Clinical information was abstracted from COG records. Event-free survival (EFS) and overall survival (OS) were calculated and compared between groups based on age and therapy.

Results: The 4-year EFS rate was 96.2% (95% confidence interval, 92%-100%), and the OS rate was 100%; EFS and OS did not statistically significantly differ with the age at diagnosis (<48 vs ≥48 months; P = .37) or treatment (EE4A vs observation only; P = .55). Six events were reported. Three patients developed contralateral tumors and did not otherwise relapse; none of these had nephrogenic rests or a recognized predisposition syndrome. Three patients developed metastatic recurrence; all 3 had received EE4A as their primary therapy after nephrectomy.

Conclusions: These findings demonstrate an excellent outcome for stage I EFHWTs with >95% EFS and OS. These data support the utility of investigating the treatment of stage I EFHWTs with observation alone after nephrectomy.

Trial registration: ClinicalTrials.gov NCT00898365.

Keywords: Wilms tumor; epithelial predominant; nephroblastoma.

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Conflict of interest statement

Conflict of Interest Statement: The authors have nothing to disclose.

Figures

Figure 1.
Figure 1.
Event-free and overall survival curves for all Stage 1 epithelial predominant favorable histology Wilms tumor (EFHWT) patients
Figure 2:
Figure 2:
Histologic appearance of different categories of EFHWT (Hematoxylin and Eosin). A. Tumor classified as EFHWT with exclusively epithelial tubular features. B. Tumor classified as EFHWT with features of metanephric adenoma. C. Tumor classified as EFHWT showing poorly differentiated epithelial features with intermixed undifferentiated (blastemal) cells comprising less than 1/3 of the tumor. D. Relapse tumor histology showing blastemal predominance, taken from a patient with EFHWT at diagnosis who was treated with EE4A
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