Outcome analysis of stage I epithelial-predominant favorable-histology Wilms tumors: A report from Children's Oncology Group study AREN03B2. Cancer 2020 Jun 15;126(12):2866-2871
Date
04/09/2020Pubmed ID
32267967Pubmed Central ID
PMC7717680DOI
10.1002/cncr.32855Scopus ID
2-s2.0-85082933704 (requires institutional sign-in at Scopus site) 17 CitationsAbstract
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.
Author List
Parsons LN, Mullen EA, Geller JI, Chi YY, Khanna G, Glick RD, Aldrink JH, Vallance KL, Kim Y, Fernandez CV, Dome JS, Perlman EJAuthor
Lauren Parsons MD Associate Professor in the Pathology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Antineoplastic Combined Chemotherapy ProtocolsChild
Child, Preschool
Disease-Free Survival
Epithelial Cells
Female
Humans
Infant
Kidney Neoplasms
Male
Neoplasm Recurrence, Local
Nephrectomy
Retrospective Studies
Wilms Tumor