The evolution of rhabdomyosarcoma (RMS) therapy is a study in rational progression based on clinical trial data. It has long been recognized that pediatric oncology patients are enrolled far more frequently on clinical protocols than adults; the serial discoveries of the Intergroup Rhabdomyosarcoma Studies (IRS) took advantage of this fact and asked discrete stepwise questions based on the accumulated results of prior trials. As a result, RMS has a wealth of solid trial based data specific for the different clinical permutations of the unique histologies in specific body sites.
It is this breadth of data that makes study of RMS daunting. IRS described not only a tumor-node-metastasis staging system, but also a unique clinical grouping schema defined post-operatively. Optimal therapy for a lesion is dependent on both, distinctly modified by the lesion’s histology. However, therapy of patients with RMS may be extremely rewarding, since increasing survival has been noted in the progressive results of the IRS trials. This is why Dr. Paulino and Dr. Okcu’s contributions in this issue are invaluable.