Rhabdomyosarcoma and other soft tissue sarcomas in children

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. During the 1960s, use of surgery and radiation therapy for local control led to survival in fewer than one third of children with this malignancy. Multimodal therapy (chemotherapy, radiation therapy, and surgery), refinements in tumor staging, and better supportive care have significantly improved outcome among children with rhabdomyosarcoma, with long-term survival rates approaching 70%. In contrast, little is known about a heterogeneous group of pediatric soft tissue sarcomas collectively termed nonrhabdomyosarcoma sort tissue sarcomas (NRSTSs). Response of NRSTSs to chemotherapeutic agents has been disappointing, and long-term remissions following chemotherapy are rare. Because the value of adjuvant therapy remains unproven, surgery is the mainstay of therapy for these tumors. This article presents clinical and biological findings of the past year that have significantly contributed to our understanding of rhabdomyosarcoma and NRSTSs.