Bilateral Wilms tumor with anaplasia: A report from the Children's Oncology Group Study AREN0534.

Wilms tumor anaplasia bilateral

Journal

Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624

Informations de publication

Date de publication:
18 Apr 2024
Historique:
revised: 06 03 2024
received: 31 01 2024
accepted: 17 03 2024
medline: 19 4 2024
pubmed: 19 4 2024
entrez: 18 4 2024
Statut: aheadofprint

Résumé

The purpose of this study is to examine the outcomes in children with anaplastic bilateral Wilms tumor (BWT) from study AREN0534 in order to define potential prognostic factors and areas to target in future clinical trials. Demographic and clinical data from AREN0534 study patients with anaplasia (focal anaplasia [FA], or diffuse anaplasia [DA]) were compared. Event-free survival (EFS) and overall survival (OS) were reported using Kaplan-Meier estimation with 95% confidence bands, and differences in outcomes between FA and DA compared using log-rank tests. The impact of margin status was analyzed. Twenty-seven children who enrolled on AREN0534 had evidence of anaplasia (17 DA, 10 FA) in at least one kidney and were included in this analysis. Twenty-six (96%) had BWT. Nineteen percent had anaplastic histology in both kidneys (four of 17 DA, and one of 10 FA). Forty-six percent with BWT had bilateral nephron-sparing surgery (NSS); one child who went off protocol therapy, eventually required bilateral completion nephrectomies. Median follow-up for EFS and OS was 8.6 and 8.7 years from enrollment. Four- and 8-year EFS was 53% [95% confidence interval (CI): 34%-83%] for DA; 4-year EFS was 80% [95% CI: 59%-100%], and 8-year EFS 70% [95% CI: 47%-100%] for FA. Three out of 10 children with FA and eight out of 17 children with DA had events. EFS did not differ statistically by margin status (p = .79; HR = 0.88). Among the six children who died (five DA, one FA), all experienced prior relapse or progression within 18 months. Events in children with DA/FA in the setting of BWT occurred early. Caution should be taken about interpreting the impact of margin status outcomes in the context of contemporary multimodal therapy. Future targeted investigations in children with BWT and DA/FA are needed.

Identifiants

pubmed: 38637871
doi: 10.1002/pbc.30981
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

e30981

Subventions

Organisme : St. Baldrick's Foundation
Organisme : NCTN Operations Center
ID : U10CA180886
Organisme : NCTN Statistics & Data Center
ID : U10CA180899

Informations de copyright

© 2024 Wiley Periodicals LLC.

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Auteurs

Rodrigo L P Romao (RLP)

Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Jennifer H Aldrink (JH)

Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio, USA.

Lindsay A Renfro (LA)

Division of Biostatistics, University of Southern California and Children's Oncology Group, Los Angeles, California, USA.

Elizabeth A Mullen (EA)

Boston Children's Hospital and Dana Farber Cancer Institute, Boston, Massachusetts, USA.

Andrew J Murphy (AJ)

St Jude's Children's Research Hospital, Memphis, Tennessee, USA.

Jack Brzezinski (J)

Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Marcus M Malek (MM)

Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.

Daniel J Benedetti (DJ)

Monroe Carell Jr Children's Hospital at Vanderbilt, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

Nicholas G Cost (NG)

The Surgical Oncology Program at the Children's Hospital of Colorado, University of Colorado, Denver, Colorado, USA.

Ethan Smith (E)

Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Jeffrey S Dome (JS)

Children National Hospital, George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.

Andrew M Davidoff (AM)

St Jude's Children's Research Hospital, Memphis, Tennessee, USA.

Amy Treece (A)

Children's Hospital of Alabama, Birmingham, Alabama, USA.

Lauren N Parsons (LN)

Children's Hospital of Wisconsin, Milwaukee, Wisconsin, USA.

Conrad V Fernandez (CV)

IWK Health, Dalhousie University, Halifax, Nova Scotia, Canada.

Brett Tornwall (B)

Department of Biostatistics, University of Florida, Gainesville, Florida, USA.

Robert C Shamberger (RC)

Boston Children's Hospital and Dana Farber Cancer Institute, Boston, Massachusetts, USA.

Arnold Paulino (A)

MD Anderson Cancer Center Houston, Houston, Texas, USA.

John A Kalapurakal (JA)

Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois, USA.

James I Geller (JI)

Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Peter F Ehrlich (PF)

Section of Pediatric Surgery, C.S. Mott Children's Hospital University of Michigan, Ann Arbor, Michigan, USA.

Classifications MeSH