Extremity rhabdomyosarcoma: biological principles, staging, and treatment.

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and adolescence with 20% of the primary tumors anatomically located on extremities. It is a complicated entity that requires careful planning and coordination between the surgical oncologist and other members of the therapeutic team for successful treatment. Even with the most effective regimens more than 50% of patients will die from progressive, usually distant disease. Further progress may require new therapeutic agents or techniques. The surgical oncologist is a necessary and often prominent member of the team. Elements of the biological behavior, histopathology, clinical staging, and treatment of extremity rhabdomyosarcomas occurring in children are discussed. In particular, the importance of the alveolar subtype in determination of prognostic risk as well as new findings regarding the molecular biologic determinants of phenotypic behavior are mentioned. Finally, innovative methods of local control like regional arterial perfusion and rapid intraoperative brachytherapy are addressed.