Rhabdomyosarcoma arising from skeletal muscle is the most common soft tissue sarcoma of childhood. Approximately 20% of patients present with disseminated disease and have a poor prognosis with combined modality therapy. The remaining 80% of patients present with localized disease and cure rates with combined modality therapy range from 50% to 90% depending on histology, site of disease and the ability of surgery to completely remove all gross and microscopic disease. One rhabdomyosarcoma study conducted showed that even after complete resection of all visible cancer and post-operative radiation therapy to the surrounding tissue, as many as 80% of the children will still have tumor recurrence in other parts of the body. Given this sobering finding, treatment regimens shifted towards giving neoadjuvant systemic chemotherapy and it is the current paradigm of rhabdomyosarcoma management. Patients with recurrent rhabdomyosarcoma have a very poor prognosis and are usually treated with chemotherapy. Active agents for the treatment of recurrent or refractory rhabdomyosarcoma include Cytoxan® (cyclophosphamide), Ifex® (ifosfamide), Adriamycin® (doxorubicin), Actinomycin D® (dactinomycin), Vepesid® (etoposide), Platinol® (cisplatin) and Paraplatin® (carboplatin). There is a completed study in which VAC chemo was the standard arm – results are pending : Combination Chemotherapy With or Without Radiation Therapy in Treating Patients With Newly Diagnosed Rhabdomyosarcoma. NCT00002995
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