End stage renal disease in patients with Wilms tumor: results from the National Wilms Tumor Study Group and the United States Renal Data System.
OBJECTIVE
We sought to assess accurately the full spectrum of end stage renal disease (ESRD) in Wilms tumor survivors by combining the unique resources of the National Wilms Tumor Study Group (NWTSG) and the United States Renal Data System (USRDS), and to confirm preliminary reports of an increased incidence of ESRD in patients with the Wilms tumor-aniridia syndrome (WAGR).


METHODS
ESRD was ascertained in 5,910 patients enrolled in NWTSG studies during 1969 to 1994 by record linkage to USRDS and by direct followup. Cumulative ESRD incidence was estimated accounting for intercurrent mortality.


RESULTS
Of 115 cases of ESRD 10 (9%) were ascertained by the NWTSG alone, 13 (11%) by USRDS alone and 92 (80%) by both. Cumulative incidence of ESRD at 20 years from diagnosis of unilateral Wilms tumor was 74% for 17 patients with the Denys-Drash syndrome, 36% for 37 patients with WAGR, 7% for 125 male patients with hypospadias or cryptorchidism (genitourinary [GU] anomalies) and 0.6% for 5,347 patients with none of these conditions. The incidence of ESRD after diagnosis of bilateral Wilms tumor was 50% for the Denys-Drash syndrome (6 patients), 90% for WAGR (10), 25% for GU anomaly (25) and 12% for other (409). ESRD in patients with WAGR or GU anomalies tended to occur relatively late, often during or after adolescence.


CONCLUSIONS
The risk of ESRD is remarkably low for the majority of patients with Wilms tumor. However, those with WAGR or associated GU anomalies are at higher risk and should be screened indefinitely to facilitate prospective treatment of impaired renal function.
Breslow
Norman E
NE
Department of Biostatistics, University of Washington, Fred Hutchinson Cancer Research Center, Seattle, Washington 98195-7232, USA. norm@u.washington.edu
Collins
Allan J
AJ
Ritchey
Michael L
ML
Grigoriev
Yevgeny A
YA
Peterson
Susan M
SM
Green
Daniel M
DM
D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003131:Combined Modality Therapy; D016001:Confidence Intervals; D030321:Denys-Drash Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D005260:Female; D006801:Humans; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D008297:Male; D009392:Nephrectomy / Q000379:methods; D011336:Probability; D011379:Prognosis; D011878:Radiotherapy / Q000379:methods; D012042:Registries; D006435:Renal Dialysis / Q000379:methods; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016019:Survival Analysis; D014481:United States / Q000453:epidemiology; D017624:WAGR Syndrome / Q000175:diagnosis; D009396:Wilms Tumor / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy