Rhabdomyosarcoma

A malignant tumor of striated muscle origin, rhabdomyosarcoma (RMS) is a childhood tumor that has benefited from nearly 30 years of multimodality therapy, culminating in a >70% overall 5-year survival. Prognosis for RMS is dependent on primary tumor site, age, completeness of resection, presence and number of metastatic sites, histology, and biology of the tumor cells. Multimodality treatment is based on risk stratification according to pre-treatment stage, postoperative group, histology, and site. Unique to RMS is the concept of postoperative clinical grouping that assesses the completeness of disease resection and takes into account lymph node evaluation at both the regional and metastatic basins. At all sites, if complete operative resection of disease is accomplished, including microscopic disease, survival is improved. Therefore, the surgeon plays a vital role in determining risk stratification for treatment, local control of the primary tumor and overall outcome for the patient with RMS.