The evolution of treatment for Wilms tumor

The prognosis for children with Wilms tumor (WT) has improved dramatically as the result of advances in surgical techniques, anesthesia, and supportive care. During the last three decades, the National Wilms Tumor Study Group (NWTSG), the International Society of Pediatric Oncology (SIOP), and the United Kingdom Children's Cancer Study Group (UKCCSG) conducted sequential studies of treatments for children with WT. The National Wilms Study Group demonstrated that radiation therapy is not necessary for those with Stage I and II, favorable histology Wilms tumor, and that the dose necessary for local control for those with local Stage III disease is 1050 to 1080 cGy. Administration of actinomycin D and doxorubicin using a single dose rather than divided dose schedule produced less myelosuppression and equivalent outcomes. Loss of heterozygosity for chromosomes 1p and 16q was associated with an inferior outcome. Areas for future investigation include the role of whole lung irradiation in the treatment of those with pulmonary metastases, the use of parenchymal sparing surgical techniques for removal of Wilms tumors, and identification of minimal necessary therapy.