WILMS-TUMOR - LONG-TERM RESULTS FROM A SINGLE INSTITUTION

One hundred-twelve children with pathological diagnosis of Wilms' tumor were treated during the period 1979-1989. They were postoperatively staged as follows: stage I, 25 patients; stage II, 27 patients; stage III, 48 patients; and stage IV, 9 patients. Three patients were preoperatively classified as stage V. The National Wilms' Tumor Study (NWTS) staging system was used. Except for Stage V patients, the treatment regimen consisted of nephrectomy followed by radiotherapy and/or chemotherapy according to stage of disease and pathology. Stage I, II, and III patients with favorable histology enjoyed 94 +/- 6%, 86 +/- 8%, and 71 +/- 8% 10-year actuarial survival, respectively. Stage IV patients and those in stages I, II, and III with unfavorable histology had a 10-year actuarial survival of 36 +/- 8%. Univariate analyses proved that NWTS stages affected the 10-year actuarial disease-free survival rates but not the overall survival rates. The histopathology significantly affected the disease-free survival and the overall survival rates both in the whole group of patients and in each stage when considered separately. The same histopathology also affected the incidence of development of distant relapse but not the local recurrence rate. On the other hand, massive tumor rupture at surgery increased the incidence of local relapse but not of distant metastasis and did not affect the overall survival rates. The presence of residuum after surgery had nearly the same effect as tumor rupture on the local recurrence. It also did not increase significantly the distant relapse incidence, but it affected significantly the overall survival. Retroperitoneal lymph node involvement had a similar effect. It increased the incidence of local relapse and decreased the 10-year overall survival rate but it had a borderline effect on development of distant metastasis (P = 0.06). The age at diagnosis had also a borderline effect on disease-free survival but it had no effect, of statistical significance, on the overall survival. (C) 1994 Wiley-Liss, Inc.