ABSTRACT
Risk stratification of a newly diagnosed patient guides therapy and predicts outcome. Rhabdomyosarcoma (RMS) is stratified into high-, intermediate- and low-risk groups based on the histological subtype, tumor site, pre-treatment stage, and the post-resection/biopsy “clinical group”. Alveolar rhabdomyosarcoma occurs more frequently in older children and adolescents with a mean age of presentation between 6 and 9 years. It typically appears in the trunk and extremities. Its diagnosis confers a poorer prognosis and by definition the patient cannot be low risk. Embryonal rhabdomyosarcoma has phenotypical and biological features of embryonic skeletal muscle with a variable number of rhabdomyoblasts and primitive mesenchymal cells in various stages of myogenesis. Multimodal therapy is the mainstay of RMS management and combines surgery, radiotherapy, and chemotherapy. Surgical resection should precede other modalities if the negative margins are achievable while avoiding disfigurement and loss of the function.
