Abstract

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• BACKGROUND: Wilms' Tumor is the most common malignant neoplasm of the urinary tract in children. Since 1969, the National Wilms' Tumor Study Group (NWTSG) has contributed to improving the clinical management and outcome of children affected by Wilms' 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's Hospital of Eastern Ontario (CHEO) with a diagnosis of Wilms' Tumor between April 1990 and March 2001.

  RESULTS:

  Forty patients with Wilms' 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%.

  CONCLUSIONS:

  The presentation of Wilms' 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.