TABLE 1:

Event free survival (EFS) or relapse free survival (RFS) and overall survival (OS) for selected high-risk or relapsed Wilms tumors in COG trials.

Diffuse anaplastic Wilms tumor
	NWTS-3 and 4; Regimen DD-RT	NWTS-3 and 4; Regimen J	NWTS-5; Regimen I	AREN0321; Regimen UH-1 or UH-2 (original or revised)
Stage II	4-year RFS 40.0% 4-year OS 46.9%	4-year RFS 71.6% 4-year OS 70.1%	4-year EFS 79.2% (95% CI, 60.9–97.5%) 4-year OS 78.4% (95% CI, 60.0–96.9%)	4-year EFS 86.7% (95% CI, 68.8–100%) 4-year OS 86.2% (
Stage III	4-year RFS 33.3% 4-year OS 20.8%	4-year RFS 58.7% 4-year OS 56.3%	4-year EFS 61.3% (95% CI, 47.8–74.7%) 4-year OS 64.7% (95% CI, 51.6–77.8%)	4-year EFS 80.9% (95% CI, 65.8–96.0%) 4-year OS 88.6% (95% CI, 76.4–100%)
Stage IV	4-year RFS 0% 4-year OS 0%	4-year RFS 16.7% 4-year OS 16.7%	4-year EFS 32.1% (95% CI, 14.8–49.4%) 4-year OS 32.1% (95% CI, 14.8–49.4%)	4-year EFS 41.7% (95% CI, 19.6–63.7%) 4-year OS 49.2% (95% CI, 27.5–71.0%)
Favorable histology Wilms tumor with LOH of 1p and 16q
	NWTS-5; EE4A	NWTS-5; DD4A	AREN0532; DD4A	AREN0533; Regimen M
Stage I-II	4-year EFS 68.8% (95% CI, 55.2–82.3%) 4-year OS 91.6% (95% CI, 83.6–99.6%)	NA	4-year EFS 87.3% (95% CI, 75.1–99.5%) 4-year OS 100%	NA
Stage III-IV	NA	4-year EFS 61.3% (95% CI, 44.9–77.6%) 4-year OS 86.0% (95% CI 90.5–100%)	NA	4-year EFS 90.2% (95% CI, 81.8–98.6%) 4-year OS 96.1% (95% CI, 90.5–100%)

Relapsed favorable histology Wilms tumor
	NWTS-2 and −3 (varied, see below)	NWTS-5; Stratum B/Regimen I	NWTS-5; Stratum C
2-drug pretreated	Stage I: 3-year OS 56.6% Stage II/III: 3-year OS 42%	4-year EFS 71.1% 4-year OS 81.8%	NA
3-drug pretreated	Stage II/III: 3-year OS 26% Stage IV: 3-year OS 17.3%	NA	4-year EFS 42.3%* 4-year OS 48%*

Note: adapted from Green, 1994⁵, Daw, 2020⁴, Gundy, 2005⁷, Dix, 2019⁸, Green, 2007¹⁰, Malogolowkin, 2008¹¹

^*mainly included FHWT, but also included small portion of patients with focal anaplastic WT

RFS: relapse-free survival; OS: overall survival; EFS: event-free survival;

DD-RT: Vincristine, Actinomycin-D, Doxorubicin

Regimen J: Vincristine, Actinomycin-D, Doxorubicin, Cyclophosphamide

Regimen I: Vincristine, Doxorubicin, Cyclophosphamide alternating with Cyclophosphamide, Etoposide

Regimen UH-1: Cyclophosphamide, Carboplatin, Etoposide alternating with Vincristine, Doxorubicin, Cyclophosphamide

Regimen UH-2: Cyclophosphamide, Carboplatin, Etoposide alternating with Vincristine, Doxorubicin, Cyclophosphamide plus Vincristine, Irinotecan

EE4A: Vincristine, Actinomycin-D

DD4A: Vincristine, Actinomycin-D, Doxorubicin

Regimen M: Vincristine, Actinomycin-D, Doxorubicin alternating with Cyclophosphamide, Etoposide

NWTS-2/−3 relapse regimens: patients were retreated with different regimens, most commonly containing Vincristine, Actinomycin-D, Doxorubicin and Cyclophosphamide; Cisplatin and Etoposide were used occasionally

Stratum B/Regimen I: Vincristine, Doxorubicin, Cyclophosphamide alternating with Etoposide, Cyclophosphamide

Stratum C: Cyclophosphamide, Etoposide alternating with Carboplatin, Etoposide