Current strategies and challenges in treatment of childhood rhabdomyosarcoma

摘要

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, affecting very young patients. These tumors often cause significant functional damage because of their aggressive growth pattern. In addition, their metastatic potential can present as a complex and challenging situation. RMS can present in various anatomical sites and often pose significant obstacle in choosing local control modalities. When feasible, surgery plays an important role for initial diagnosis and complete tumor removal; delayed primary re-excision and second-look surgery after initial chemotherapy are gaining more acceptance. Because of high-metastatic risk, systemic chemotherapy is also necessary. Novel agents are emerging which may alter the disease course in high-risk disease where the cure rate is still low. Radiation therapy is an important tool in the management of RMS and has gone through significant evolution during past four decades. This review will outline treatment strategies adopted in children RMS. The primary focus will be the North American approach with attention to advancements in radiation therapy, surgical techniques, and systemic therapies.