Wilms' tumor and neuroblastoma: results of therapy

Advances in chemotherapy of Wilms' tumor, along with improved treatment modalities in radiotherapy and surgery have increased the survival rate from a 20% 2 yr survival rate 3 decades ago to an 80% plus rate within the last 10 yr period. The use of dactinomycin and vincristine accounts for the improvement in chemotherapy. The primary objective of the National Wilms' Tumor Study, activated in 1969, was to refine methods of treatment. Whether postoperative radiotherapy is necessary for treatment of patients with well encapsulated, localized lesions after what appears to be their total removal; which of the 2 chemotherapeutic agents (dactinomycin, vincristine) known to be effective against Wilms' tumor gives the better result; and whether this result can be improved by their combined use, are considered. Although there has been marked improvement in survival of children with Wilms' tumor during the last decade, this has not been the case for metastatic and nonmetastatic neuroblastoma for the same time period, primarily due to the difficulty in early diagnosis and lack of effective treatment for advanced disease. Chemotherapy, in contrast to Wilms' tumor, has not been very successful. Although results of treatment continue to be poor, advances have been made in the understanding of the disease process. A new staging system has been developed based on certain patterns of origin and clinical behavior found to affect prognosis, which takes into account the peculiarities unique for neuroblastoma. A second potential contribution has been the development of a simple, reliable 'dip stick' test for detecting the early presence of neuroblastoma.