111 renal neoplasms of childhood: A clinicopathologic study

Purpose: The aim of this study was to perform a clinicopathologic evaluatio n of a single pediatric institution renal tumor series. Most patients were treated within the frame of 3 consecutive SIOP trials, which included preop erative chemotherapy as their main feature. Methods: Medical records and diagnoses of 111 patients were reviewed. The a ssociation of pathologic features with outcome was investigated by means of the Kaplan-Meier method, the Cox model, and a logistic multivariate analys is. Comparison among different trial results was carried out. Results: In 98 patients (88%), nephroblastoma was diagnosed, followed by 6 adult-type renal tumors, 3 cystic nephromas, 2 mesoblastic nephromas, and 2 clear cell sarcomas. For nephroblastoma, a statistically significant corre lation between grade and both disease-free survival rate and 5-year surviva l rate, and between stage and overall survival rate was shown. Lymph node i nvolvement, local relapse, nephrogenic rests, and older age at presentation appeared to be less important prognostic factors. Tumor spillage was very sensitive to chemo or radiotherapy. No significant difference in outcome wa s observed among different trials. Conclusions: Wilms' tumor was the most frequent neoplasm and resulted in a 5-year cure rate of 90%. Clinical course was influenced mainly by diffuse a naplasia and, to a minor extent, by lymph node involvement. Because some tu mors followed an unpredictable course, it is likely that also other biologi cal factors played a significant role. Copyright (C) 2001 by W.B. Saunders Company.