Treatment of stage I anaplastic Wilms' tumour

a report from the Children's Oncology Group AREN0321 study

AREN0321 Study Committee

Research output: Contribution to journalArticle

Abstract

Background: In the fifth National Wilms Tumor Study (NWTS-5), the 4-year event-free survival (EFS) and overall survival (OS) estimates for 29 patients with stage I focal (n = 10) or diffuse (n = 19) anaplastic Wilms' tumour (AWT) treated with vincristine and dactinomycin without flank radiation were 69.5% and 82.6%, respectively. The Children's Oncology Group AREN0321 study evaluated whether adding doxorubicin and flank radiation improves survival for these patients. Patients and methods: Tumour histology and stage were confirmed by real-time central pathology, surgery and radiology review. The patients received 25 weeks of vincristine, dactinomycin and doxorubicin (cumulative dose 150 mg/m2) with flank radiation (1080 cGy). We retrospectively analysed outcomes of all patients with stage I AWT enrolled in NWTSs 1–5 and AREN0321 with respect to treatment regimens. Results: Eighteen patients with stage I AWT (8 focal and 10 diffuse) were enrolled on AREN0321. With a median follow-up of 4.6 years, the 4-year EFS and OS were 100%. One patient with diffuse AWT had pulmonary relapse 4.12 years after diagnosis. In the 112 patients with stage I AWT treated in NWTSs 1–5 and AREN0321, the EFS was significantly improved with doxorubicin treatment (p = 0.01; 4-year EFS: 97.2% [95% confidence interval {CI}: 91.3–100] vs. 77.5% [95% CI: 67.6–87.4]) but not by flank radiation (p = 0.15). Conclusions: Treatment of stage I AWT with vincristine, dactinomycin, doxorubicin and flank radiation in AREN0321 yielded excellent survival outcomes. Retrospective analysis of AREN0321 and NWTS patients suggests that doxorubicin had a greater contribution to the excellent outcomes than radiation.

Original languageEnglish (US)
Pages (from-to)58-66
Number of pages9
JournalEuropean Journal of Cancer
Volume118
DOIs
StatePublished - Sep 1 2019

Fingerprint

Wilms Tumor
Doxorubicin
Radiation
Disease-Free Survival
Dactinomycin
Vincristine
Therapeutics
Survival
Confidence Intervals
Radiology
Histology
Pathology
Recurrence
Lung

Keywords

  • Dactinomycin
  • Diffuse anaplasia
  • Doxorubicin
  • Focal anaplasia
  • Outcome
  • Radiation
  • Stage I
  • Treatment
  • Vincristine
  • Wilms tumour

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

@article{785c347cb6b14cf3b23d1d39c3b1c348,
title = "Treatment of stage I anaplastic Wilms' tumour: a report from the Children's Oncology Group AREN0321 study",
abstract = "Background: In the fifth National Wilms Tumor Study (NWTS-5), the 4-year event-free survival (EFS) and overall survival (OS) estimates for 29 patients with stage I focal (n = 10) or diffuse (n = 19) anaplastic Wilms' tumour (AWT) treated with vincristine and dactinomycin without flank radiation were 69.5{\%} and 82.6{\%}, respectively. The Children's Oncology Group AREN0321 study evaluated whether adding doxorubicin and flank radiation improves survival for these patients. Patients and methods: Tumour histology and stage were confirmed by real-time central pathology, surgery and radiology review. The patients received 25 weeks of vincristine, dactinomycin and doxorubicin (cumulative dose 150 mg/m2) with flank radiation (1080 cGy). We retrospectively analysed outcomes of all patients with stage I AWT enrolled in NWTSs 1–5 and AREN0321 with respect to treatment regimens. Results: Eighteen patients with stage I AWT (8 focal and 10 diffuse) were enrolled on AREN0321. With a median follow-up of 4.6 years, the 4-year EFS and OS were 100{\%}. One patient with diffuse AWT had pulmonary relapse 4.12 years after diagnosis. In the 112 patients with stage I AWT treated in NWTSs 1–5 and AREN0321, the EFS was significantly improved with doxorubicin treatment (p = 0.01; 4-year EFS: 97.2{\%} [95{\%} confidence interval {CI}: 91.3–100] vs. 77.5{\%} [95{\%} CI: 67.6–87.4]) but not by flank radiation (p = 0.15). Conclusions: Treatment of stage I AWT with vincristine, dactinomycin, doxorubicin and flank radiation in AREN0321 yielded excellent survival outcomes. Retrospective analysis of AREN0321 and NWTS patients suggests that doxorubicin had a greater contribution to the excellent outcomes than radiation.",
keywords = "Dactinomycin, Diffuse anaplasia, Doxorubicin, Focal anaplasia, Outcome, Radiation, Stage I, Treatment, Vincristine, Wilms tumour",
author = "{AREN0321 Study Committee} and Daw, {Najat C.} and Chi, {Yueh Yun} and Yeonil Kim and Mullen, {Elizabeth A.} and Kalapurakal, {John A.} and Jing Tian and Kalapurakal, {John A} and Geller, {James I.} and Perlman, {Elizabeth J.} and Ehrlich, {Peter F.} and Perlman, {Elizabeth J} and Grundy, {Paul E.} and Fernandez, {Conrad V.} and Dome, {Jeffrey S.}",
year = "2019",
month = "9",
day = "1",
doi = "10.1016/j.ejca.2019.05.033",
language = "English (US)",
volume = "118",
pages = "58--66",
journal = "European Journal of Cancer",
issn = "0959-8049",
publisher = "Elsevier Limited",

}

Treatment of stage I anaplastic Wilms' tumour : a report from the Children's Oncology Group AREN0321 study. / AREN0321 Study Committee.

In: European Journal of Cancer, Vol. 118, 01.09.2019, p. 58-66.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Treatment of stage I anaplastic Wilms' tumour

T2 - a report from the Children's Oncology Group AREN0321 study

AU - AREN0321 Study Committee

AU - Daw, Najat C.

AU - Chi, Yueh Yun

AU - Kim, Yeonil

AU - Mullen, Elizabeth A.

AU - Kalapurakal, John A.

AU - Tian, Jing

AU - Kalapurakal, John A

AU - Geller, James I.

AU - Perlman, Elizabeth J.

AU - Ehrlich, Peter F.

AU - Perlman, Elizabeth J

AU - Grundy, Paul E.

AU - Fernandez, Conrad V.

AU - Dome, Jeffrey S.

PY - 2019/9/1

Y1 - 2019/9/1

N2 - Background: In the fifth National Wilms Tumor Study (NWTS-5), the 4-year event-free survival (EFS) and overall survival (OS) estimates for 29 patients with stage I focal (n = 10) or diffuse (n = 19) anaplastic Wilms' tumour (AWT) treated with vincristine and dactinomycin without flank radiation were 69.5% and 82.6%, respectively. The Children's Oncology Group AREN0321 study evaluated whether adding doxorubicin and flank radiation improves survival for these patients. Patients and methods: Tumour histology and stage were confirmed by real-time central pathology, surgery and radiology review. The patients received 25 weeks of vincristine, dactinomycin and doxorubicin (cumulative dose 150 mg/m2) with flank radiation (1080 cGy). We retrospectively analysed outcomes of all patients with stage I AWT enrolled in NWTSs 1–5 and AREN0321 with respect to treatment regimens. Results: Eighteen patients with stage I AWT (8 focal and 10 diffuse) were enrolled on AREN0321. With a median follow-up of 4.6 years, the 4-year EFS and OS were 100%. One patient with diffuse AWT had pulmonary relapse 4.12 years after diagnosis. In the 112 patients with stage I AWT treated in NWTSs 1–5 and AREN0321, the EFS was significantly improved with doxorubicin treatment (p = 0.01; 4-year EFS: 97.2% [95% confidence interval {CI}: 91.3–100] vs. 77.5% [95% CI: 67.6–87.4]) but not by flank radiation (p = 0.15). Conclusions: Treatment of stage I AWT with vincristine, dactinomycin, doxorubicin and flank radiation in AREN0321 yielded excellent survival outcomes. Retrospective analysis of AREN0321 and NWTS patients suggests that doxorubicin had a greater contribution to the excellent outcomes than radiation.

AB - Background: In the fifth National Wilms Tumor Study (NWTS-5), the 4-year event-free survival (EFS) and overall survival (OS) estimates for 29 patients with stage I focal (n = 10) or diffuse (n = 19) anaplastic Wilms' tumour (AWT) treated with vincristine and dactinomycin without flank radiation were 69.5% and 82.6%, respectively. The Children's Oncology Group AREN0321 study evaluated whether adding doxorubicin and flank radiation improves survival for these patients. Patients and methods: Tumour histology and stage were confirmed by real-time central pathology, surgery and radiology review. The patients received 25 weeks of vincristine, dactinomycin and doxorubicin (cumulative dose 150 mg/m2) with flank radiation (1080 cGy). We retrospectively analysed outcomes of all patients with stage I AWT enrolled in NWTSs 1–5 and AREN0321 with respect to treatment regimens. Results: Eighteen patients with stage I AWT (8 focal and 10 diffuse) were enrolled on AREN0321. With a median follow-up of 4.6 years, the 4-year EFS and OS were 100%. One patient with diffuse AWT had pulmonary relapse 4.12 years after diagnosis. In the 112 patients with stage I AWT treated in NWTSs 1–5 and AREN0321, the EFS was significantly improved with doxorubicin treatment (p = 0.01; 4-year EFS: 97.2% [95% confidence interval {CI}: 91.3–100] vs. 77.5% [95% CI: 67.6–87.4]) but not by flank radiation (p = 0.15). Conclusions: Treatment of stage I AWT with vincristine, dactinomycin, doxorubicin and flank radiation in AREN0321 yielded excellent survival outcomes. Retrospective analysis of AREN0321 and NWTS patients suggests that doxorubicin had a greater contribution to the excellent outcomes than radiation.

KW - Dactinomycin

KW - Diffuse anaplasia

KW - Doxorubicin

KW - Focal anaplasia

KW - Outcome

KW - Radiation

KW - Stage I

KW - Treatment

KW - Vincristine

KW - Wilms tumour

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U2 - 10.1016/j.ejca.2019.05.033

DO - 10.1016/j.ejca.2019.05.033

M3 - Article

VL - 118

SP - 58

EP - 66

JO - European Journal of Cancer

JF - European Journal of Cancer

SN - 0959-8049

ER -