Abstract :
The management of children with soft tissue sarcomas has changed and survival has improved markedly during the past three decades. This is, in large part, due to the multi-modal approach to pediatric malignancies; decreasing the need for radical surgical procedures, while improving survival and decreasing morbidity. The management of patients with rhabdomyosarcomas (RMS) and nonrhabdomyosarcomas soft-tissue sarcomas (NRSTS) as well as neurofibromatosis and related tumors is best accomplished in an organized and systematic fashion with the surgeon involved early in the planning, diagnostic, and treatment phases. Cooperative trials for rhabdomyosarcoma, as part of the Intergroup Rhabdomyosarcoma Study, have led to improved survival with less radical surgery. Ongoing trials of the Soft Tissue Sarcoma Group of the Children’s Oncology Group are under way. Since these tumors are rare, cooperative trials are essential to answering the remaining questions regarding extent of surgery and adjuvant therapy.
