Molecular and surgical advances in pediatric tumors.

Abstract

Before the 1950s, less than a third of children with Wilms’ tumor, rhabdomyosarcoma (RMS), or neuroblastoma survived. Because of the rarity of these tumors, no single institution could collect enough patients for randomized trials. In the United States, the pediatric cancer study groups joined forces to create the National Wilms’ Tumor Study Group (NWTS) in 1969 and the Intergroup Rhabdomyosarcoma Study Committee (IRS) in 1972. Protocols to treat neuroblastoma have been developed by both the Children’s Cancer Group (CCG) and the Pediatric Oncology Group (POG). The multinational Societe Internationale d’Oncologie Pediatrique (SIOP) studies form a European counterpart to these groups. The protocols used by these groups that employ multimodality therapy including multiagent chemotherapy, have led to the current long-term survival approaching 40% in neuroblastoma [1], 70% in RMS [2], and 95% in Wilms’ tumor [3]. Each group continues to modify risk-adapted therapy based on identified prognostic factors. Recent identifications of molecular level differences may allow for further stratification of risk and adaptation of therapy to maximize survival while minimizing therapy.