Wilms' tumor, a model for chemosensitive tumors in pediatric patients

Pediatric malignancies are rare disorders with a number of distinctive feat ures as compared to adult malignancies, including spontaneously rapid growt h and extreme sensitivity to radiation therapy and chemotherapy. Actinomyci n D, vincristine, and doxorubicin are the main agents used to treat Wilms' tumor, followed closely by ifosfamide and, above all, etoposide and carbopl atin. However, radiation therapy adversely affects normal growth, doxorubic in is cardiotoxic, and etoposide can cause severe leukemia. The challenge i s therefore to select, for each case or group of cases, the combination tha t is both most likely to ensure recovery and least likely to cause sequelae or delayed complications. Vast international therapeutic trials have provi ded the data needed to meet this challenge successfully. A full recovery wi th no sequelae can now be achieved in 90% of Wilms' tumor patients, with fi rst-line chemotherapy being a substantial contributor to this favorable out come. Other pediatric tumors can be treated successfully based on the same principles.