Table 1.

Cumulative incidence of ESRD and overall survival at 20 years from diagnosis of Wilms tumor

Congenital malformation group	No. of patients	Mean FU time (years)	No. of renal failures observed	Incidence of ESRD at 20 years from diagnosis (95% CI)	No. of deaths observed	Survival at 20 years from diagnosis (95% CI)
Cohort I: Unilateral Wilms tumor
DDS	17	10.6	12	74% (45-89%)	4	68% (31-88%)
WAGR	37	14.0	11	36% (18-55%)	8	78% (55-90%)
GU anomalies	125	14.2	4	6.7% (1.9-16%)	10	93% (86-96%)
None	5,347	12.7	28	0.6% (0.4-1.0%)	730	85% (84-86%)
Total	5,526	12.7	55	1.3% (0.9%-1.7%)	752	85% (84-86%)
Cohort II:Bilateral Wilms tumor
DDS	6	13.4	3	50% (11-80%)	1	83% (27-98%)
WAGR	10	11.1	5	90% (47-99%)	3	56% (19-85%)
GU anomalies	25	15.2	8	25% (8-47%)	6	79% (57-91%)
None	409	11.3	44	11.5% (8-15%)	115	70% (64-74%)
Total	450	11.5	60	15% (11-19%)	125	70% (65-75%)

Abbreviations: CI = confidence interval; DDS = Denys-Drash syndrome; FU = follow-up; WAGR = Wilms tumor-aniridia syndrome