Issues in the local control of rhabdomyosarcoma

Abstract

Background: Rhabdomyosarcoma (RMS) is a heterogeneous disease consisting of several different histologies arising from a variety of anatomic sites. Approximately half of the children who die of this tumor have failure at the primary site of involvement, making local control an important component of therapy.

Procedure: Published literature and newly analyzed data from the Intergroup Rhabdomyosarcoma Study Group (IRSG) regarding local control of RMS were reviewed. Information regarding the role of various local control modalities for different primary disease sites is presented along with new directions for clinical research.

Results: Local control rates for RMS average 80% for group III tumors, with large variations seen for different anatomic sites. Important gains in functional outcome for certain sites such as gynecologic system and bladder/prostate have been achieved by optimizing the use of the various treatment modalities. Local control at other sites such as the chest and extremities remains a problem.

Conclusions: Advances in surgical and radiotherapy techniques coupled with multiagent chemotherapy are providing improved local control with decreasing morbidity. Optimal outcome is dependent on close collaboration between surgical, radiotherapy, and pediatric oncology specialists.