More than 7,000 new cases of cancer are diagnosed in children under the age of 15 each year in the United States.1 Despite important improvements in care, one-third of these patients die from either disease progression or complications of aggressive therapy. In the more developed countries of the world, cancer is the leading cause of death by disease in children after the first year of life. Childhood leukemia is the most common pediatric malignancy, followed by brain tumors and lymphoma. The next large group of tumors are the solid tumors of childhood, which represent an area that concerns pediatric surgeons. Although survival has improved for children with certain pediatric neoplasms, including Wilms' tumor, acute lymphocytic leukemia, non-Hodgkin's lymphoma, and Hodgkin's disease, some tumors such as neuroblastoma remain difficult to cure.2-4

The overall improvement in outcomes in childhood solid tumors has been related to the development of national multidisciplinary cooperative studies carried out in children's cancer centers and the availability of pediatric medical and surgical cancer specialists who devote much of their activity to the management of infants and children with cancer. This has permitted the development of well-designed tumor treatment protocols characterized by uniform staging criteria; diagnostic resources for assessment of pathology (histology); uniform laboratory methods for measuring tumor markers, cytokines, oncogenes, and other important biologic and genetic factors; and careful data collection. Frequent review and prompt statistical analysis of data allow for early beneficial changes in care and the deletion of therapy that results in adverse effects. The excellent results achieved by cooperative pediatric tumor study groups such as the Children's Cancer Group, Pediatric Oncology Group, Intergroup Rhabdomyosarcoma Study, and the National Wilms' Tumor Study clearly reflect the advantages of a multidisciplinary approach to care. Recently, all of these organizations have merged into a single national pediatric cancer organization called the Children's Oncology Group.

Important advances in the biologic study of cancer and its genetic basis have led to a number of observations that impact directly on the management of childhood solid tumors.4-12 Identification of specific genes, oncogenes, tumor markers, and other biologic and pathologic factors plays an important role in both staging and clarifying the risk categorization of individual patients. Treatment of the patient is influenced by the recognition of specific risk factors. This knowledge has resulted in a change in the approach to care based not only on staging criteria, but also on risk-based management. This concept uses various risk factors as predictors of outcomes.

The purpose of this report is to update the reader on information regarding the genetic basis and current risk-management protocols for children with the more common pediatric solid tumors, including Wilms' tumor, rhabdomyosarcoma, and neuroblastoma.