BACKGROUND: Wilms&apos; Tumor is the most common malignant neoplasm of the urinary tract in children. Since 1969, the National Wilms&apos; Tumor Study Group (NWTSG) has contributed to improving the clinical management and outcome of children affected by Wilms&apos; Tumor. We have managed our patients according to NWTSG protocols and report our results herein. METHODS: Retrospective chart review of consecutive patients presenting at the Children&apos;s Hospital of Eastern Ontario (CHEO) with a diagnosis of Wilms&apos; Tumor between April 1990 and March 2001.
Forty patients with Wilms&apos; Tumor (18 M/22 F) were diagnosed at CHEO during this interval. Mean age at diagnosis was 28.5 months. The most common presenting feature was a palpable abdominal mass in 85%. An overgrowth syndrome was noted in 10%. Metastatic disease was present at diagnosis in 20%, with the lungs (75%) the most common site of involvement. Distribution of clinical stage: I = 40%, II = 20%, III = 20%, IV = 15%, V = 5%. Favorable histology (FH) was diagnosed in 82.5%, anaplasia in 12.5% and clear cell sarcoma (CCSK) in 5%. Nephrogenic rests were present in 43% (perilobar (PL) = 20%, intralobar (IL) = 18%, PL + IL = 5%). Recurrence of disease occurred in 10%, with lungs (100%) the most frequent site of relapse. Survival was significantly greater in stage I disease and in those patients with FH. Patient 4-year survival data: stage I (FH), 100%; stage II (FH), 80%; stage III (FH), 100%, stage IV (FH), 67%; stages I-IV (CCSK), 100%; stage V, 0%. Overall 4-year survival rates of patients with FH were 92% and of all patients in the study was 86%.
The presentation of Wilms&apos; Tumor at our institution mirrors that described in the literature. Importantly, by following NWTSG protocols we have achieved an outcome of overall 4-year patient survival comparable to the "gold standard". This demonstrates the utility of the NWTSG protocols as regards patient management of this relatively uncommon disease.